DOI: 10.1006/exnr.1998.7006. Recent Advances on the Pathogenesis of Huntington's Disease

Recent Advances on the Pathogenesis of Huntington's Disease

10.1006/exnr.1998.7006

Crossref journal-article

Elsevier BV

Experimental Neurology (78)

Bibliography

PetersÃ©n, Ã., Mani, K., & Brundin, P. (1999). Recent Advances on the Pathogenesis of Huntingtonâs Disease. Experimental Neurology, 157(1), 1â18.

Authors 3

Åsa Petersén (first)
Kevin Mani (additional)
Patrik Brundin (additional)

References 168 Referenced 116

10.1016/0166-2236(95)93943-R / Trends Neurosci. / Genetics and molecular biology of Huntington's disease by Albin (1995)
10.1097/00001756-199808030-00001 / Neuroreport / 3-Nitroproprionic acid's lethal triplet: Cooperative pathways of neurodegeneration by Alexi (1998)
10.1007/BF02257483 / Somat. Cell Mol. Genet. / Structure and expression of the Huntington's disease gene: Evidence against simple inactivation due to an expanded CAG repeat by Ambrose (1994)
10.1038/ng1095-115 / Nature Genet. / HEAT repeats in the Huntington's disease protein by Andrade (1995)
10.1038/ng0893-398 / Nature Genet. / The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease by Andrew (1993)
10.1016/0896-6273(95)90186-8 / Neuron / Glutamate-induced neuronal death: A succession of necrosis or apoptosis depending on mitochondrial function by Ankarcrona (1995)
10.1016/0014-5793(96)00959-3 / FEBS Lett. / Calcineurin and mitochondrial function in glutamate induced neuronal apoptosis by Ankarcrona (1996)
10.1016/0896-6273(95)90106-X / Neuron / CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain by Aronin (1995)
10.1097/00005072-199704000-00013 / J. Neuropathol. Exp. Neurol. / Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington's disease-An in situ hybridization study by Arzberger (1997)
10.1212/WNL.44.6.1137 / Neurology / CAG repeat size and clinical presentation in Huntington's disease by Ashizawa (1994)
10.1002/ana.410420213 / Ann. Neurol. / Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease by Augood (1997)
10.1073/pnas.93.10.5037 / Proc. Natl. Acad. Sci. USA / Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin by Bao (1996)
10.1038/321168a0 / Nature / Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid by Beal (1986)
10.1523/JNEUROSCI.11-06-01649.1991 / J. Neurosci. / Chronic quinolinic acid lesions in rats closely resemble Huntington's disease by Beal (1989)
10.1002/ana.410310202 / Ann. Neurol. / Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses by Beal (1992)
10.1111/j.1471-4159.1993.tb03633.x / J. Neurochem. / Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate by Beal (1993)
10.1016/0166-2236(93)90117-5 / Trends Neurosci. / Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases by Beal (1993)
10.1002/ana.410380304 / Ann. Neurol. / Aging, energy and oxidative stress in neurodegenerative diseases by Beal (1995)
10.1006/nbdi.1998.0168 / Neurobiol. Dis. / Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions andIT15 by Becher (1998)
10.1097/00001756-199502000-00034 / Neuroreport / 3-nitroproprionic acid induces apoptosis in cultured striatal and cortical neurons by Behrens (1995)
10.1097/00001756-199707070-00031 / Neuroreport / Fast transport and retrograde movement of huntingtin and HAP1 in axons by Block-Galarza (1997)
10.1007/BF00691525 / Acta Neuropathol. / Neuropathological changes of the nucleus accumbens in Huntington's chorea by Bots (1987)
10.1212/WNL.46.2.527 / Neurology / Trinucleotide repeat length and clinical progression in Huntington's disease by Brandt (1996)
10.1002/ana.410380604 / Ann. Neurol. / Neural apoptosis by Bredesen (1995)
10.1016/0896-6273(91)90374-9 / Neuron / Nitric oxide synthase protein and mRNA are discretely localized in neuronal populations of the mammalian CNS together with NADPH diaphorase by Bredt (1991)
10.1038/nm0396-347 / Nature Med. / Huntingtin and DRPLA proteins selectively interact with the enzyme GADPH by Burke (1996)
10.1016/S0306-4522(98)00129-8 / Neuroscience / Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum by Butterworth (1998)
10.1016/0304-3940(93)90448-T / Neurosci. Lett. / Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: A pathological correlate of dementia by Cammarata (1993)
10.1007/s004390050273 / Hum. Genet. / Transglutaminase activity is related to CAG repeat length in patients with Huntington's disease by Cariello (1996)
10.1073/pnas.95.11.6480 / Proc. Natl. Acad. Sci. USA / Altered brain neurotransmittor receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene by Cha (1998)
{'key': '10.1006/exnr.1998.7006_RF31', 'first-page': '380.5', 'article-title': "Neuroanatomical features of a transgenic model of Huntington's disease", 'author': 'Chase', 'year': '1998', 'journal-title': 'Soc. Neurosci. Abstr.'} / Soc. Neurosci. Abstr. / Neuroanatomical features of a transgenic model of Huntington's disease by Chase (1998)
10.1001/archneur.1995.00540320021009 / Arch. Neurol. / Correlations between triplet repeat expansion and clinical features in Huntington's disease by Claes (1995)
10.1093/hmg/6.9.1519 / Hum. Mol. Genet. / Huntingtin-associated protein 1 (HAP1) binds to a Trio-like polypeptide, with a rac1 guanine nucleotide exchange factor domain by Colomer (1997)
10.1038/263244a0 / Nature / Lesion of striatal neurones with kainic acid provides a model for Huntington's chorea by Coyle (1976)
10.1126/science.7901908 / Science / Oxidative stress, glutamate, and neurodegenerative disorders by Coyle (1993)
10.1016/S0092-8674(00)80513-9 / Cell / Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation by Davies (1997)
10.1016/S0140-6736(97)08360-8 / Lancet / Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions by Davies (1998)
10.1073/pnas.88.17.7797 / Proc. Natl. Acad. Sci. USA / Nitric oxide synthase and neuronal NADPH diaphorase are identical in brain and peripheral tissue by Dawson (1991)
10.1523/JNEUROSCI.13-06-02651.1993 / J. Neurosci. / Mechanisms of nitric oxide-mediated neurotoxicity in primary brain cultures by Dawson (1993)
10.1523/JNEUROSCI.16-08-02479.1996 / J. Neurosci. / Resistance to neurotoxicity in cortical cultures from neuronal nitric oxide synthase-deficient mice by Dawson (1996)
10.1093/hmg/5.8.1093 / Hum. Mol. Genet. / Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation by De Rooij (1996)
{'key': '10.1006/exnr.1998.7006_RF42', 'first-page': '1040', 'article-title': 'Apoptosis in the brain', 'volume': '146', 'author': 'Dickson', 'year': '1995', 'journal-title': 'Am. J. Pathol.'} / Am. J. Pathol. / Apoptosis in the brain by Dickson (1995)
10.1016/0896-6273(95)90346-1 / Neuron / Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons by DiFiglia (1995)
10.1126/science.277.5334.1990 / Science / Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain by DiFiglia (1997)
10.1177/107385849800400113 / Neuroscientist / Mitochondrial DNA and diseases of the nervous system: The spectrum by DiMauro (1998)
{'key': '10.1006/exnr.1998.7006_RF46', 'first-page': '319', 'article-title': 'Excitatory amino acid receptors and neurodegeneration', 'volume': '50', 'author': 'Doble', 'year': '1995', 'journal-title': 'Therapie'} / Therapie / Excitatory amino acid receptors and neurodegeneration by Doble (1995)
10.1523/JNEUROSCI.15-10-06377.1995 / J. Neurosci. / Mitochondrial production of reactive oxygen species in cortical nertons following exposure toNd by Dugan (1995)
10.1126/science.7618107 / Science / Inactivation of the mouse Huntington's disease gene homologHdh by Duyao (1995)
10.1097/00001756-199505090-00026 / Neuroreport / In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes by Dragunow (1995)
10.1093/hmg/6.13.2205 / Hum. Mol. Genet. / Huntingtin-associated protein 1 (HAP1) interacts with the p150 Glued subunit of dynactin by Engelender (1997)
10.1002/ajmg.1320180115 / Am. J. Hum. Genet. / The natural history of Huntington's disease: Possible role of “aging genes.” by Farrer (1984)
{'key': '10.1006/exnr.1998.7006_RF52', 'first-page': '528', 'article-title': "Inverse relationship between age at onset of Huntington's disease and paternal age suggests involvement of genetic imprinting", 'volume': '50', 'author': 'Farrer', 'year': '1992', 'journal-title': 'Am. J. Hum. Genet.'} / Am. J. Hum. Genet. / Inverse relationship between age at onset of Huntington's disease and paternal age suggests involvement of genetic imprinting by Farrer (1992)
10.1097/00005072-198701000-00002 / J. Neuropathol. Exp. Neurol. / Morphologic and histochemical characteristics of a spared subset of neurons in Huntington's disease by Ferrante (1987)
10.1523/JNEUROSCI.17-09-03052.1997 / J. Neurosci. / Heterogenous topographic and cellular distribution of huntingtin expression in the normal human neostriatum by Ferrante (1997)
{'key': '10.1006/exnr.1998.7006_RF55', 'first-page': '119', 'article-title': 'Searching for function of tissue transglutaminase: Its possible involvement in biochemical pathway of programmed cell death', 'volume': '231', 'author': 'Fesus', 'year': '1988', 'journal-title': 'Adv. Expl. Biol.'} / Adv. Expl. Biol. / Searching for function of tissue transglutaminase: Its possible involvement in biochemical pathway of programmed cell death by Fesus (1988)
10.1007/BF00995383 / Neurochem. Res. / Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration by Fujita (1995)
10.1083/jcb.119.3.493 / J. Cell Biol. / Identification of programmed cell deathin situ by Gavrieli (1992)
10.1124/mol.52.1.6 / Mol. Pharmacol. / High affinity glutamate transporters: Regulation of expression and activity by Gegelashvili (1997)
10.1126/science.8303297 / Science / Transcriptional activation modulated by homopolymeric glutamine and proline stretches by Gerber (1994)
10.1016/0166-2236(92)90355-C / Trends Neurosci. / The neostriatal mosaic: Multiple levels of compartmental organization by Gerfen (1992)
10.1038/ng0896-442 / Nature Genet. / Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract by Goldberg (1996)
10.1523/JNEUROSCI.18-06-02040.1998 / J. Neurosci. / Manganese superoxide dismutase protects nNOS neurons from NMDA and nitric oxide-mediated neurotoxicity by Gonzalez-Zulueta (1998)
10.1002/ana.410420507 / Ann. Neurol. / Differential distribution of the normal and mutated forms of huntingtin in the human brain by Gourfinkel-An (1997)
10.1001/archneur.1992.00530350075022 / Arch. Neurol. / Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease by Grafton (1992)
10.1046/j.1471-4159.1995.64010430.x / J. Neurochem. / Characterization of the excitotoxic potential of the reversible succinate dehydrogenase inhibitor malonate by Greene (1995)
10.1002/ana.410390317 / Ann. Neurol. / Mitochondrial defect in Huntington's disease caudate nucleus by Gu (1996)
10.1038/306234a0 / Nature / A polymorphic DNA marker genetically linked to Huntington's disease by Gusella (1983)
10.1001/archneur.1993.00540110037003 / Arch. Neurol. / Molecular genetics of Huntington's disease by Gusella (1993)
10.1073/pnas.92.19.8710 / Proc. Natl. Acad. Sci. USA / Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein anti-bodies by Gutekunst (1995)
10.1111/j.1399-0004.1998.tb02687.x / Clin. Genet. / The fatal attraction of polyglutamine-containing proteins by Hackam (1998)
10.1016/0736-5748(93)90060-Q / Int. J. Dev. Neurosci. / Cellular transglutaminases in neural development by Hand (1993)
10.1007/BF00194305 / Hum. Genet. / The epidemiology of Huntington's disease by Harper (1992)
10.1016/0304-3940(91)90583-F / Neurosci. Lett. / Neuronal loss in layer V and VI of cerebral cortex in Huntington's disease by Hedreen (1991)
10.1097/00005072-199501000-00013 / J. Neuropathol. Exp. Neurol. / Early loss of neostriatal striosome neurones in Huntington's disease by Hedreen (1995)
{'key': '10.1006/exnr.1998.7006_RF75', 'first-page': '127.1', 'article-title': 'YAC transgenic mice expressing mutant human huntington show deficits in hippocampal long-term potentiation', 'author': 'Hodgson', 'year': '1998', 'journal-title': 'Soc. Neurosci. Abstr.'} / Soc. Neurosci. Abstr. / YAC transgenic mice expressing mutant human huntington show deficits in hippocampal long-term potentiation by Hodgson (1998)
10.1093/hmg/2.12.2069 / Hum. Mol. Genet. / Characterization and localization of the Huntington's disease gene product by Hoogeveen (1993)
10.1073/pnas.88.7.2811 / Proc. Natl. Acad. Sci. USA / Neuronal NADPH diaphorase is a nitric oxide synthase by Hope (1991)
10.1016/0306-4522(94)00494-P / Neuroscience / Quinolinic acid-induced increases in calbindin D28K immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease by Huang (1995)
10.1016/0092-8674(93)90585-E / Cell (1993)
10.1212/WNL.43.12.2689 / Neurology / Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy by Jenkins (1993)
10.1023/A:1005340302695 / J. Inherit. Metab. Dis. / Huntington's disease: Advances in molecular and cell biology by Jones (1997)
10.1016/S1097-2765(00)80059-3 / Mol. Cell / Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine by Kahlem (1998)
10.1074/jbc.271.32.19385 / J. Biol. Chem. / Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme by Kalchman (1996)
10.1038/ng0597-44 / Nature Genet. / HIP1,S. cerevisiae Sla2p, by Kalchman (1997)
10.1038/bjc.1972.33 / Br. J. Cancer / Apoptosis: A basic biological phenomenon with wide ranging implications in tissue kinetics by Kerr (1972)
10.1126/science.2875522 / Science / Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity by Koh (1986)
10.1093/hmg/5.9.1311 / Hum. Mol. Genet. / Spinocerbellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase by Koshy (1996)
10.1016/0166-2236(87)90120-2 / Trends Neurosci. / Patterns of cell loss in Huntington's disease by Kowall (1987)
{'key': '10.1006/exnr.1998.7006_RF89', 'first-page': '735', 'article-title': "Covariate-dependent age-at-onset distributions for Huntington's disease", 'volume': '49', 'author': 'Krawczak', 'year': '1991', 'journal-title': 'Am. J. Hum. Genet.'} / Am. J. Hum. Genet. / Covariate-dependent age-at-onset distributions for Huntington's disease by Krawczak (1991)
10.1212/WNL.43.8.1593 / Neurology / AMPA and NMDA binding sites in the hypothalamic lateral tuberal nucleus: Implications for Huntington's disease by Kremer (1993)
10.1097/00005072-199007000-00002 / J. Neuropathol. Exp. Neurol. / Atrophy of the hypothalamic lateral tuberal nucleus in Huntington's Disease by Kremer (1990)
10.1001/archneur.1992.00530280029014 / Arch. Neurol. / Weight loss in Huntington's disease by Kremer (1992)
10.1093/brain/113.5.1405 / Brain / Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease by Kuwert (1990)
{'key': '10.1006/exnr.1998.7006_RF94', 'first-page': '380.8', 'article-title': "Development and characterization of a novel transgenic model of Huntington's disease which recapitulates features of the human illness", 'author': 'Laforet', 'year': '1998', 'journal-title': 'Soc. Neurosci. Abstr.'} / Soc. Neurosci. Abstr. / Development and characterization of a novel transgenic model of Huntington's disease which recapitulates features of the human illness by Laforet (1998)
10.1006/excr.1997.4026 / Exp. Cell Res. / Apoptosis, excitotoxicity, and neuropathology by Leist (1998)
{'key': '10.1006/exnr.1998.7006_RF96', 'first-page': '380.7', 'article-title': "Enhanced sensitivity to glutamate receptor activation in mouse models of Huntington's disease", 'author': 'Levine', 'year': '1998', 'journal-title': 'Soc. Neurosci. Abstr.'} / Soc. Neurosci. Abstr. / Enhanced sensitivity to glutamate receptor activation in mouse models of Huntington's disease by Levine (1998)
10.1093/hmg/7.5.777 / Hum. Mol. Genet. / Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats by Li (1998)
10.1038/378398a0 / Nature / A huntingtin-associated protein enriched in brain with implications for pathology by Li (1995)
10.1073/pnas.93.10.4839 / Proc. Natl. Acad. Sci. USA / Huntingtin-associated protein (HAP1): Discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase by Li (1996)
10.1038/364626a0 / Nature / A redox-based mechanism for neuroprotective and neurodestructive effects of nitric oxide and related nitroso-compounds by Lipton (1993)
10.1056/NEJM199403033300907 / N. Engl. J. Med. / Excitatory amino acids as a final common pathway for neurological disorders by Lipton (1994)
10.1017/S0317167100032212 / Can. J. Neurol. Sci. / 3-Nitropropionic acid-exogenous animal toxin and possible human striatal toxin by Ludolph (1991)
10.1016/S0959-4388(96)80097-3 / Curr. Opin. Neurobiol. / Huntington's disease: Translating a CAG repeat into a pathogenetic model by MacDonald (1996)
{'key': '10.1006/exnr.1998.7006_RF104', 'series-title': "Huntington's Disease", 'article-title': "The neurobiology of Huntington's disease", 'author': 'MacMillan', 'year': '1996'} / Huntington's Disease / The neurobiology of Huntington's disease by MacMillan (1996)
10.1016/S0092-8674(00)81369-0 / Cell / Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice by Mangiarini (1996)
10.1038/ng0297-197 / Nature Genet. / Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation by Mangiarini (1997)
10.1016/0896-6273(92)90087-T / Neuron / Nitric oxide-induced blockade of NMDA receptors by Manzoni (1992)
10.1046/j.1471-4159.1997.69010285.x / J. Neurochem. / Iodoacetate produces striatal excitotoxic lesions by Matthews (1997)
10.1016/0006-8993(78)90941-1 / Brain Res. / Kainate-induced degeneration of neostriatal neurons: Dependency upon corticostriatal tract by McGeer (1978)
10.1016/S0306-4522(97)00594-0 / Neuroscience / Methylmalonate toxicity in primary neuronal cultures by McLaughlin (1998)
10.1093/hmg/6.5.775 / Hum. Mol. Genet. / Reduced penetrance of the Huntington's disease mutation by McNeil (1997)
{'key': '10.1006/exnr.1998.7006_RF112', 'first-page': '380.4', 'article-title': 'Conditional inactivation of huntingtin in transgenic mice', 'author': 'Metzler', 'year': '1998', 'journal-title': 'Soc. Neurosci. Abstr.'} / Soc. Neurosci. Abstr. / Conditional inactivation of huntingtin in transgenic mice by Metzler (1998)
10.1146/annurev.pa.29.040189.002053 / Annu. Rev. Pharmacol. Toxicol. / The excitatory amino acid receptors: Their classes, pharmacology, and distinct properties in the function of the central nervous system by Monaghan (1989)
10.1161/01.CIR.91.2.513 / Circulation / Unstable triplet repeat diseases by Monckton (1995)
{'key': '10.1006/exnr.1998.7006_RF115', 'series-title': "Huntington's Disease", 'first-page': '73', 'article-title': "Psychiatric aspects of Huntington's disease", 'author': 'Morris', 'year': '1996'} / Huntington's Disease / Psychiatric aspects of Huntington's disease by Morris (1996)
{'key': '10.1006/exnr.1998.7006_RF116', 'series-title': 'Protection of Striatal and Nigral Neurons by Trophic and Antioxidant Mechanisms: A Study on in Vitro and in Vivo Brain Insult Models and Neural Transplants', 'author': 'Nakao', 'year': '1996'} / Protection of Striatal and Nigral Neurons by Trophic and Antioxidant Mechanisms: A Study on in Vitro and in Vivo Brain Insult Models and Neural Transplants by Nakao (1996)
10.1016/S0306-4522(96)00223-0 / Neuroscience / Effects of alpha-phenyl-tert by Nakao (1997)
10.1016/0092-8674(95)90542-1 / Cell / Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes by Nasir (1995)
10.1016/S0968-0004(97)01085-2 / Trends Biochem. Sci. / Caspases: Killer proteins by Nicholson (1997)
10.1016/0304-3940(96)12794-4 / Neurosci. Lett. / In sity end-labeling detects necrosis of hippocampal pyramidal cells induced by kainic acid by Nishiyama (1996)
10.1016/0006-8993(88)90765-2 / Brain Res. / Glutamate becomes neurotoxic via theNd by Novelli (1988)
10.1126/science.164.3880.719 / Science / Brain lesion, obesity and other disturbances in mice treated with monosodium glutamate by Olney (1969)
10.1016/S0896-6273(00)80943-5 / Neuron / Intranuclear inclusions of expanded polyglutamine protein in spinocerbellar ataxia type 3 by Paulson (1997)
10.1523/JNEUROSCI.15-05-03775.1995 / J. Neurosci. / Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models by Portera-Cailliau (1995)
10.1073/pnas.91.12.5355 / Proc. Natl. Acad. Sci. USA / Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases by Perutz (1994)
{'key': '10.1006/exnr.1998.7006_RF126', 'series-title': "Huntington's Disease", 'first-page': '31', 'article-title': "The clinical neurology of Huntington's disease", 'author': 'Quarrell', 'year': '1996'} / Huntington's Disease / The clinical neurology of Huntington's disease by Quarrell (1996)
{'key': '10.1006/exnr.1998.7006_RF127', 'first-page': '593', 'article-title': 'Anticipation and instability of IT-15 (CAG)N', 'volume': '57', 'author': 'Ranen', 'year': '1995', 'journal-title': 'Am. J. Hum. Genet.'} / Am. J. Hum. Genet. / Anticipation and instability of IT-15 (CAG)N by Ranen (1995)
10.1073/pnas.85.15.5733 / Proc. Natl. Acad. Sci. USA / Differential loss of striatal projection neurons in Huntington's disease by Reiner (1988)
10.1016/S0960-9822(02)70711-2 / Curr. Biol. / Rho: Theme and variations by Ridley (1996)
10.1083/jcb.130.5.1093 / J. Cell Biol. / A mutation in glyceraldehyde-3-phosphate dehydrogenase alters endocytosis in CHO cells by Robbins (1995)
{'key': '10.1006/exnr.1998.7006_RF131', 'series-title': "Advances in Neurology, Vol. 23, Huntington's Disease", 'first-page': '95', 'article-title': "Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations", 'author': 'Roizin', 'year': '1979'} / Advances in Neurology, Vol. 23, Huntington's Disease / Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations by Roizin (1979)
{'key': '10.1006/exnr.1998.7006_RF132', 'first-page': '31', 'article-title': "Nuclear membrane indentations in Huntington's chorea", 'volume': '61', 'author': 'Ross', 'year': '1983', 'journal-title': 'J. Neurol. Sci.'} / J. Neurol. Sci. / Nuclear membrane indentations in Huntington's chorea by Ross (1983)
10.1016/0896-6273(95)90138-8 / Neuron / When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases by Ross (1995)
10.1016/S0896-6273(00)80405-5 / Neuron / Intranuclear neuronal inclusions: A common pathogenetic mechanism for glutamine-repeat neurodegenerative diseases by Ross (1997)
10.5694/j.1326-5377.1981.tb135681.x / Med. J. Aust. / Body weight and dietary factors on Huntington's disease patients compared with matched controls by Sanberg (1981)
10.1002/ana.410420411 / Ann. Neurol. / Huntingtin localization in brains of normal and Huntington's disease patients by Sapp (1997)
10.1016/S0092-8674(00)80514-0 / Cell / Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo by Scherzinger (1997)
10.1016/0197-4580(94)90128-7 / Neurobiol. Aging / Metabotropic glutamate receptors and neuronal degenerative disorders by Schoepp (1994)
10.1046/j.1471-4159.1995.64052239.x / J. Neurochem. / Involvement of free radicals in excitotoxicityin vivo by Schulz (1995)
{'key': '10.1006/exnr.1998.7006_RF140', 'first-page': '110', 'article-title': 'Free radicals scavengers protect dopaminergic cell lines from apoptosis by complex I inhibitors', 'volume': '777', 'author': 'Seaton', 'year': '1997', 'journal-title': 'Brain Res.'} / Brain Res. / Free radicals scavengers protect dopaminergic cell lines from apoptosis by complex I inhibitors by Seaton (1997)
10.1016/S0140-6736(88)92073-9 / Lancet / Loss of matrix calcium-binding protein-containing neurons in Huntington's disease by Seto-Oshima (1988)
10.1016/0896-6273(95)90345-3 / Neuron / Widespread expression of Huntington's disease gene (IT15) protein product by Sharp (1995)
10.1006/nbdi.1996.0002 / Neurobiol. Dis. / Neurobiology of Huntington's disease by Sharp (1996)
10.1006/nbdi.1998.0177 / Neurobiol. Dis. / Neuronal death in cultured murine cortical cells is induced by inhibition of GAPDH and triosephosphate isomerase by Sheline (1998)
10.1016/0014-5793(96)00989-1 / FEBS Lett. / Why are mitochondria involved in apoptosis? Permeability transition pores and apoptosis as selective mechanisms to eliminate superoxide-producing mitochondria and cell by Skulachev (1996)
10.1038/ng0893-393 / Nature Genet. / Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease by Snell (1993)
10.1038/ng1193-259 / Nature Genet. / Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues by Strong (1993)
10.1084/jem.184.4.1331 / J. Exp. Med. / Bcl-2 inhibits the mitochondrial release of an apoptogenic protease by Susin (1996)
10.1038/ng0494-409 / Nature Genet. / Somatic and gonadal mosaicism of the Huntington's disease gene CAG repeat in brain and sperm by Telenius (1994)
10.1097/00005072-197404000-00008 / J. Neuropathol. Exp. Neurol. / Studies on brain biopsies of patients with Huntington's chorea by Tellez-Nagel (1974)
10.1006/exnr.1995.1029 / Exp. Neurol. / DNA end labeling (TUNEL) in Huntington's disease and other neuropathological conditions by Thomas (1995)
10.1126/science.281.5381.1312 / Science / Caspases: Enemies within by Thornberry (1998)
10.1136/jmg.31.5.377 / J. Med. Genet. / Instability of CAG repeats in Huntington's disease: Relation to parental transmission and age of onset by Trottier (1994)
10.1038/ng0595-104 / Nature Genet. / Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form by Trottier (1995)
10.1016/S0169-328X(97)00205-2 / Mol. Brain Res. / Huntington's disease gene product, huntingtin, associates with microtubules in vitro by Tukamoto (1997)
10.1006/exnr.1998.6832 / Exp. Neurol. / Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways by Velier (1998)
10.1097/00005072-199805000-00001 / J. Neuropathol. Exp. Neurol. / Huntington's disease by Vonsattel (1998)
10.1136/jnnp.64.6.758 / J. Neurol. Neurosurg. Psychiatry / Genetic polymorphisms adjacent to the CAG repeat influence clinical features at onset in Huntington's disease by Vuillaume (1998)
10.1006/exnr.1994.1081 / Exp. Neurol. / Selective loss of [33 by Wagster (1994)
10.1093/hmg/6.3.487 / Hum. Mol. Genet. / HIP-1: A huntingtin interacting protein isolated by the yeast two-hybrid system by Wanker (1997)
10.1002/ana.410400110 / Ann. Neurol. / Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease by Weeks (1996)
10.1111/j.1750-3639.1997.tb00897.x / Brain Pathol. / Toward understanding the molecular pathology of Huntington's disease by Wellington (1997)
10.1038/326194a0 / Nature / Homozygotes for Huntington's disease by Wexler (1987)
{'key': '10.1006/exnr.1998.7006_RF164', 'series-title': 'Cell Death in Biology and Pathology', 'first-page': '9', 'article-title': 'Cell death: A new classification separating apoptosis from necrosis', 'author': 'Wyllie', 'year': '1981'} / Cell Death in Biology and Pathology / Cell death: A new classification separating apoptosis from necrosis by Wyllie (1981)
10.1126/science.2841762 / Science / NMDA receptor losses in putamen from patients with Huntington's disease by Young (1988)
10.1016/0092-8674(93)90485-9 / Cell / TheC. elegans by Yuan (1993)
10.1023/A:1022694131572 / J. Bionerg. Biomembr. / Mitochondrial implication in accidental and programmed cell death: Apoptosis and necrosis by Zamzami (1997)
10.1038/ng1095-155 / Nature Genet. / Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue by Zeitlin (1995)

Dates

Type	When
Created	22 years, 10 months ago (Oct. 6, 2002, 7 p.m.)
Deposited	5 years, 8 months ago (Dec. 17, 2019, 8:24 a.m.)
Indexed	1 month, 1 week ago (July 25, 2025, 6:46 a.m.)
Issued	26 years, 4 months ago (May 1, 1999)
Published	26 years, 4 months ago (May 1, 1999)
Published Print	26 years, 4 months ago (May 1, 1999)

Funders 0

None

BibTeX

@article{Peters_n_1999, title={Recent Advances on the Pathogenesis of Huntington’s Disease}, volume={157}, ISSN={0014-4886}, url={http://dx.doi.org/10.1006/exnr.1998.7006}, DOI={10.1006/exnr.1998.7006}, number={1}, journal={Experimental Neurology}, publisher={Elsevier BV}, author={Petersén, Åsa and Mani, Kevin and Brundin, Patrik}, year={1999}, month=may, pages={1–18} }

JSON

{
  "indexed": {
    "date-parts": [
      [
        2025,
        7,
        25
      ]
    ],
    "date-time": "2025-07-25T10:46:56Z",
    "timestamp": 1753440416328
  },
  "reference-count": 168,
  "publisher": "Elsevier BV",
  "issue": "1",
  "license": [
    {
      "start": {
        "date-parts": [
          [
            1999,
            5,
            1
          ]
        ],
        "date-time": "1999-05-01T00:00:00Z",
        "timestamp": 925516800000
      },
      "content-version": "tdm",
      "delay-in-days": 0,
      "URL": "https://www.elsevier.com/tdm/userlicense/1.0/"
    }
  ],
  "content-domain": {
    "domain": [],
    "crossmark-restriction": false
  },
  "published-print": {
    "date-parts": [
      [
        1999,
        5
      ]
    ]
  },
  "DOI": "10.1006/exnr.1998.7006",
  "type": "journal-article",
  "created": {
    "date-parts": [
      [
        2002,
        10,
        6
      ]
    ],
    "date-time": "2002-10-06T23:00:46Z",
    "timestamp": 1033945246000
  },
  "page": "1-18",
  "source": "Crossref",
  "is-referenced-by-count": 116,
  "title": "Recent Advances on the Pathogenesis of Huntington's Disease",
  "prefix": "10.1006",
  "volume": "157",
  "author": [
    {
      "given": "\u00c5sa",
      "family": "Peters\u00e9n",
      "sequence": "first",
      "affiliation": []
    },
    {
      "given": "Kevin",
      "family": "Mani",
      "sequence": "additional",
      "affiliation": []
    },
    {
      "given": "Patrik",
      "family": "Brundin",
      "sequence": "additional",
      "affiliation": []
    }
  ],
  "member": "78",
  "reference": [
    {
      "key": "10.1006/exnr.1998.7006_RF1",
      "doi-asserted-by": "crossref",
      "first-page": "11",
      "DOI": "10.1016/0166-2236(95)93943-R",
      "article-title": "Genetics and molecular biology of Huntington's disease",
      "volume": "18",
      "author": "Albin",
      "year": "1995",
      "journal-title": "Trends Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF2",
      "doi-asserted-by": "crossref",
      "first-page": "R57",
      "DOI": "10.1097/00001756-199808030-00001",
      "article-title": "3-Nitroproprionic acid's lethal triplet: Cooperative pathways of neurodegeneration",
      "volume": "9",
      "author": "Alexi",
      "year": "1998",
      "journal-title": "Neuroreport"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF3",
      "doi-asserted-by": "crossref",
      "first-page": "27",
      "DOI": "10.1007/BF02257483",
      "article-title": "Structure and expression of the Huntington's disease gene: Evidence against simple inactivation due to an expanded CAG repeat",
      "volume": "20",
      "author": "Ambrose",
      "year": "1994",
      "journal-title": "Somat. Cell Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF4",
      "doi-asserted-by": "crossref",
      "first-page": "115",
      "DOI": "10.1038/ng1095-115",
      "article-title": "HEAT repeats in the Huntington's disease protein",
      "volume": "11",
      "author": "Andrade",
      "year": "1995",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF5",
      "doi-asserted-by": "crossref",
      "first-page": "398",
      "DOI": "10.1038/ng0893-398",
      "article-title": "The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease",
      "volume": "4",
      "author": "Andrew",
      "year": "1993",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF6",
      "doi-asserted-by": "crossref",
      "first-page": "961",
      "DOI": "10.1016/0896-6273(95)90186-8",
      "article-title": "Glutamate-induced neuronal death: A succession of necrosis or apoptosis depending on mitochondrial function",
      "volume": "15",
      "author": "Ankarcrona",
      "year": "1995",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF7",
      "doi-asserted-by": "crossref",
      "first-page": "321",
      "DOI": "10.1016/0014-5793(96)00959-3",
      "article-title": "Calcineurin and mitochondrial function in glutamate induced neuronal apoptosis",
      "volume": "394",
      "author": "Ankarcrona",
      "year": "1996",
      "journal-title": "FEBS Lett."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF8",
      "doi-asserted-by": "crossref",
      "first-page": "1193",
      "DOI": "10.1016/0896-6273(95)90106-X",
      "article-title": "CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain",
      "volume": "15",
      "author": "Aronin",
      "year": "1995",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF9",
      "doi-asserted-by": "crossref",
      "first-page": "440",
      "DOI": "10.1097/00005072-199704000-00013",
      "article-title": "Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington's disease-An in situ hybridization study",
      "volume": "56",
      "author": "Arzberger",
      "year": "1997",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF10",
      "doi-asserted-by": "crossref",
      "first-page": "1137",
      "DOI": "10.1212/WNL.44.6.1137",
      "article-title": "CAG repeat size and clinical presentation in Huntington's disease",
      "volume": "44",
      "author": "Ashizawa",
      "year": "1994",
      "journal-title": "Neurology"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF11",
      "doi-asserted-by": "crossref",
      "first-page": "215",
      "DOI": "10.1002/ana.410420213",
      "article-title": "Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease",
      "volume": "42",
      "author": "Augood",
      "year": "1997",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF12",
      "doi-asserted-by": "crossref",
      "first-page": "5037",
      "DOI": "10.1073/pnas.93.10.5037",
      "article-title": "Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin",
      "volume": "93",
      "author": "Bao",
      "year": "1996",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF13",
      "doi-asserted-by": "crossref",
      "first-page": "168",
      "DOI": "10.1038/321168a0",
      "article-title": "Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid",
      "volume": "321",
      "author": "Beal",
      "year": "1986",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF14",
      "doi-asserted-by": "crossref",
      "first-page": "1649",
      "DOI": "10.1523/JNEUROSCI.11-06-01649.1991",
      "article-title": "Chronic quinolinic acid lesions in rats closely resemble Huntington's disease",
      "volume": "11",
      "author": "Beal",
      "year": "1989",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF15",
      "doi-asserted-by": "crossref",
      "first-page": "119",
      "DOI": "10.1002/ana.410310202",
      "article-title": "Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses",
      "volume": "31",
      "author": "Beal",
      "year": "1992",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF16",
      "doi-asserted-by": "crossref",
      "first-page": "1147",
      "DOI": "10.1111/j.1471-4159.1993.tb03633.x",
      "article-title": "Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate",
      "volume": "61",
      "author": "Beal",
      "year": "1993",
      "journal-title": "J. Neurochem."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF17",
      "doi-asserted-by": "crossref",
      "first-page": "125",
      "DOI": "10.1016/0166-2236(93)90117-5",
      "article-title": "Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases",
      "volume": "16",
      "author": "Beal",
      "year": "1993",
      "journal-title": "Trends Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF18",
      "doi-asserted-by": "crossref",
      "first-page": "357",
      "DOI": "10.1002/ana.410380304",
      "article-title": "Aging, energy and oxidative stress in neurodegenerative diseases",
      "volume": "38",
      "author": "Beal",
      "year": "1995",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF19",
      "doi-asserted-by": "crossref",
      "first-page": "387",
      "DOI": "10.1006/nbdi.1998.0168",
      "article-title": "Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions andIT15",
      "volume": "4",
      "author": "Becher",
      "year": "1998",
      "journal-title": "Neurobiol. Dis."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF20",
      "doi-asserted-by": "crossref",
      "first-page": "545",
      "DOI": "10.1097/00001756-199502000-00034",
      "article-title": "3-nitroproprionic acid induces apoptosis in cultured striatal and cortical neurons",
      "volume": "6",
      "author": "Behrens",
      "year": "1995",
      "journal-title": "Neuroreport"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF21",
      "doi-asserted-by": "crossref",
      "first-page": "2247",
      "DOI": "10.1097/00001756-199707070-00031",
      "article-title": "Fast transport and retrograde movement of huntingtin and HAP1 in axons",
      "volume": "8",
      "author": "Block-Galarza",
      "year": "1997",
      "journal-title": "Neuroreport"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF22",
      "doi-asserted-by": "crossref",
      "first-page": "21",
      "DOI": "10.1007/BF00691525",
      "article-title": "Neuropathological changes of the nucleus accumbens in Huntington's chorea",
      "volume": "55",
      "author": "Bots",
      "year": "1987",
      "journal-title": "Acta Neuropathol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF23",
      "doi-asserted-by": "crossref",
      "first-page": "527",
      "DOI": "10.1212/WNL.46.2.527",
      "article-title": "Trinucleotide repeat length and clinical progression in Huntington's disease",
      "volume": "46",
      "author": "Brandt",
      "year": "1996",
      "journal-title": "Neurology"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF24",
      "doi-asserted-by": "crossref",
      "first-page": "839",
      "DOI": "10.1002/ana.410380604",
      "article-title": "Neural apoptosis",
      "volume": "38",
      "author": "Bredesen",
      "year": "1995",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF25",
      "doi-asserted-by": "crossref",
      "first-page": "615",
      "DOI": "10.1016/0896-6273(91)90374-9",
      "article-title": "Nitric oxide synthase protein and mRNA are discretely localized in neuronal populations of the mammalian CNS together with NADPH diaphorase",
      "volume": "7",
      "author": "Bredt",
      "year": "1991",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF26",
      "doi-asserted-by": "crossref",
      "first-page": "347",
      "DOI": "10.1038/nm0396-347",
      "article-title": "Huntingtin and DRPLA proteins selectively interact with the enzyme GADPH",
      "volume": "2",
      "author": "Burke",
      "year": "1996",
      "journal-title": "Nature Med."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF27",
      "doi-asserted-by": "crossref",
      "first-page": "49",
      "DOI": "10.1016/S0306-4522(98)00129-8",
      "article-title": "Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum",
      "volume": "87",
      "author": "Butterworth",
      "year": "1998",
      "journal-title": "Neuroscience"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF28",
      "doi-asserted-by": "crossref",
      "first-page": "96",
      "DOI": "10.1016/0304-3940(93)90448-T",
      "article-title": "Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: A pathological correlate of dementia",
      "volume": "156",
      "author": "Cammarata",
      "year": "1993",
      "journal-title": "Neurosci. Lett."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF29",
      "doi-asserted-by": "crossref",
      "first-page": "633",
      "DOI": "10.1007/s004390050273",
      "article-title": "Transglutaminase activity is related to CAG repeat length in patients with Huntington's disease",
      "volume": "98",
      "author": "Cariello",
      "year": "1996",
      "journal-title": "Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF30",
      "doi-asserted-by": "crossref",
      "first-page": "6480",
      "DOI": "10.1073/pnas.95.11.6480",
      "article-title": "Altered brain neurotransmittor receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene",
      "volume": "95",
      "author": "Cha",
      "year": "1998",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF31",
      "first-page": "380.5",
      "article-title": "Neuroanatomical features of a transgenic model of Huntington's disease",
      "author": "Chase",
      "year": "1998",
      "journal-title": "Soc. Neurosci. Abstr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF32",
      "doi-asserted-by": "crossref",
      "first-page": "749",
      "DOI": "10.1001/archneur.1995.00540320021009",
      "article-title": "Correlations between triplet repeat expansion and clinical features in Huntington's disease",
      "volume": "52",
      "author": "Claes",
      "year": "1995",
      "journal-title": "Arch. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF33",
      "doi-asserted-by": "crossref",
      "first-page": "1519",
      "DOI": "10.1093/hmg/6.9.1519",
      "article-title": "Huntingtin-associated protein 1 (HAP1) binds to a Trio-like polypeptide, with a rac1 guanine nucleotide exchange factor domain",
      "volume": "6",
      "author": "Colomer",
      "year": "1997",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF34",
      "doi-asserted-by": "crossref",
      "first-page": "244",
      "DOI": "10.1038/263244a0",
      "article-title": "Lesion of striatal neurones with kainic acid provides a model for Huntington's chorea",
      "volume": "263",
      "author": "Coyle",
      "year": "1976",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF35",
      "doi-asserted-by": "crossref",
      "first-page": "689",
      "DOI": "10.1126/science.7901908",
      "article-title": "Oxidative stress, glutamate, and neurodegenerative disorders",
      "volume": "262",
      "author": "Coyle",
      "year": "1993",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF36",
      "doi-asserted-by": "crossref",
      "first-page": "537",
      "DOI": "10.1016/S0092-8674(00)80513-9",
      "article-title": "Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation",
      "volume": "90",
      "author": "Davies",
      "year": "1997",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF37",
      "doi-asserted-by": "crossref",
      "first-page": "131",
      "DOI": "10.1016/S0140-6736(97)08360-8",
      "article-title": "Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions",
      "volume": "351",
      "author": "Davies",
      "year": "1998",
      "journal-title": "Lancet"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF38",
      "doi-asserted-by": "crossref",
      "first-page": "7797",
      "DOI": "10.1073/pnas.88.17.7797",
      "article-title": "Nitric oxide synthase and neuronal NADPH diaphorase are identical in brain and peripheral tissue",
      "volume": "88",
      "author": "Dawson",
      "year": "1991",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF39",
      "doi-asserted-by": "crossref",
      "first-page": "2651",
      "DOI": "10.1523/JNEUROSCI.13-06-02651.1993",
      "article-title": "Mechanisms of nitric oxide-mediated neurotoxicity in primary brain cultures",
      "volume": "13",
      "author": "Dawson",
      "year": "1993",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF40",
      "doi-asserted-by": "crossref",
      "first-page": "2479",
      "DOI": "10.1523/JNEUROSCI.16-08-02479.1996",
      "article-title": "Resistance to neurotoxicity in cortical cultures from neuronal nitric oxide synthase-deficient mice",
      "volume": "16",
      "author": "Dawson",
      "year": "1996",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF41",
      "doi-asserted-by": "crossref",
      "first-page": "1093",
      "DOI": "10.1093/hmg/5.8.1093",
      "article-title": "Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation",
      "volume": "5",
      "author": "De Rooij",
      "year": "1996",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF42",
      "first-page": "1040",
      "article-title": "Apoptosis in the brain",
      "volume": "146",
      "author": "Dickson",
      "year": "1995",
      "journal-title": "Am. J. Pathol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF43",
      "doi-asserted-by": "crossref",
      "first-page": "1075",
      "DOI": "10.1016/0896-6273(95)90346-1",
      "article-title": "Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons",
      "volume": "14",
      "author": "DiFiglia",
      "year": "1995",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF44",
      "doi-asserted-by": "crossref",
      "first-page": "1990",
      "DOI": "10.1126/science.277.5334.1990",
      "article-title": "Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain",
      "volume": "277",
      "author": "DiFiglia",
      "year": "1997",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF45",
      "doi-asserted-by": "crossref",
      "first-page": "53",
      "DOI": "10.1177/107385849800400113",
      "article-title": "Mitochondrial DNA and diseases of the nervous system: The spectrum",
      "volume": "4",
      "author": "DiMauro",
      "year": "1998",
      "journal-title": "Neuroscientist"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF46",
      "first-page": "319",
      "article-title": "Excitatory amino acid receptors and neurodegeneration",
      "volume": "50",
      "author": "Doble",
      "year": "1995",
      "journal-title": "Therapie"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF47",
      "doi-asserted-by": "crossref",
      "first-page": "6377",
      "DOI": "10.1523/JNEUROSCI.15-10-06377.1995",
      "article-title": "Mitochondrial production of reactive oxygen species in cortical nertons following exposure toNd",
      "volume": "15",
      "author": "Dugan",
      "year": "1995",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF48",
      "doi-asserted-by": "crossref",
      "first-page": "407",
      "DOI": "10.1126/science.7618107",
      "article-title": "Inactivation of the mouse Huntington's disease gene homologHdh",
      "volume": "269",
      "author": "Duyao",
      "year": "1995",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF49",
      "doi-asserted-by": "crossref",
      "first-page": "1053",
      "DOI": "10.1097/00001756-199505090-00026",
      "article-title": "In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes",
      "volume": "6",
      "author": "Dragunow",
      "year": "1995",
      "journal-title": "Neuroreport"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF50",
      "doi-asserted-by": "crossref",
      "first-page": "2205",
      "DOI": "10.1093/hmg/6.13.2205",
      "article-title": "Huntingtin-associated protein 1 (HAP1) interacts with the p150 Glued subunit of dynactin",
      "volume": "6",
      "author": "Engelender",
      "year": "1997",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF51",
      "doi-asserted-by": "crossref",
      "first-page": "115",
      "DOI": "10.1002/ajmg.1320180115",
      "article-title": "The natural history of Huntington's disease: Possible role of \u201caging genes.\u201d",
      "volume": "18",
      "author": "Farrer",
      "year": "1984",
      "journal-title": "Am. J. Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF52",
      "first-page": "528",
      "article-title": "Inverse relationship between age at onset of Huntington's disease and paternal age suggests involvement of genetic imprinting",
      "volume": "50",
      "author": "Farrer",
      "year": "1992",
      "journal-title": "Am. J. Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF53",
      "doi-asserted-by": "crossref",
      "first-page": "12",
      "DOI": "10.1097/00005072-198701000-00002",
      "article-title": "Morphologic and histochemical characteristics of a spared subset of neurons in Huntington's disease",
      "volume": "46",
      "author": "Ferrante",
      "year": "1987",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF54",
      "doi-asserted-by": "crossref",
      "first-page": "3052",
      "DOI": "10.1523/JNEUROSCI.17-09-03052.1997",
      "article-title": "Heterogenous topographic and cellular distribution of huntingtin expression in the normal human neostriatum",
      "volume": "17",
      "author": "Ferrante",
      "year": "1997",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF55",
      "first-page": "119",
      "article-title": "Searching for function of tissue transglutaminase: Its possible involvement in biochemical pathway of programmed cell death",
      "volume": "231",
      "author": "Fesus",
      "year": "1988",
      "journal-title": "Adv. Expl. Biol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF56",
      "doi-asserted-by": "crossref",
      "first-page": "1195",
      "DOI": "10.1007/BF00995383",
      "article-title": "Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration",
      "volume": "20",
      "author": "Fujita",
      "year": "1995",
      "journal-title": "Neurochem. Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF57",
      "doi-asserted-by": "crossref",
      "first-page": "493",
      "DOI": "10.1083/jcb.119.3.493",
      "article-title": "Identification of programmed cell deathin situ",
      "volume": "119",
      "author": "Gavrieli",
      "year": "1992",
      "journal-title": "J. Cell Biol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF58",
      "doi-asserted-by": "crossref",
      "first-page": "6",
      "DOI": "10.1124/mol.52.1.6",
      "article-title": "High affinity glutamate transporters: Regulation of expression and activity",
      "volume": "52",
      "author": "Gegelashvili",
      "year": "1997",
      "journal-title": "Mol. Pharmacol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF59",
      "doi-asserted-by": "crossref",
      "first-page": "808",
      "DOI": "10.1126/science.8303297",
      "article-title": "Transcriptional activation modulated by homopolymeric glutamine and proline stretches",
      "volume": "263",
      "author": "Gerber",
      "year": "1994",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF60",
      "doi-asserted-by": "crossref",
      "first-page": "133",
      "DOI": "10.1016/0166-2236(92)90355-C",
      "article-title": "The neostriatal mosaic: Multiple levels of compartmental organization",
      "volume": "15",
      "author": "Gerfen",
      "year": "1992",
      "journal-title": "Trends Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF61",
      "doi-asserted-by": "crossref",
      "first-page": "442",
      "DOI": "10.1038/ng0896-442",
      "article-title": "Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract",
      "volume": "13",
      "author": "Goldberg",
      "year": "1996",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF62",
      "doi-asserted-by": "crossref",
      "first-page": "2040",
      "DOI": "10.1523/JNEUROSCI.18-06-02040.1998",
      "article-title": "Manganese superoxide dismutase protects nNOS neurons from NMDA and nitric oxide-mediated neurotoxicity",
      "volume": "18",
      "author": "Gonzalez-Zulueta",
      "year": "1998",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF63",
      "doi-asserted-by": "crossref",
      "first-page": "712",
      "DOI": "10.1002/ana.410420507",
      "article-title": "Differential distribution of the normal and mutated forms of huntingtin in the human brain",
      "volume": "42",
      "author": "Gourfinkel-An",
      "year": "1997",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF64",
      "doi-asserted-by": "crossref",
      "first-page": "1161",
      "DOI": "10.1001/archneur.1992.00530350075022",
      "article-title": "Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease",
      "volume": "49",
      "author": "Grafton",
      "year": "1992",
      "journal-title": "Arch. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF65",
      "doi-asserted-by": "crossref",
      "first-page": "430",
      "DOI": "10.1046/j.1471-4159.1995.64010430.x",
      "article-title": "Characterization of the excitotoxic potential of the reversible succinate dehydrogenase inhibitor malonate",
      "volume": "64",
      "author": "Greene",
      "year": "1995",
      "journal-title": "J. Neurochem."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF66",
      "doi-asserted-by": "crossref",
      "first-page": "385",
      "DOI": "10.1002/ana.410390317",
      "article-title": "Mitochondrial defect in Huntington's disease caudate nucleus",
      "volume": "39",
      "author": "Gu",
      "year": "1996",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF67",
      "doi-asserted-by": "crossref",
      "first-page": "234",
      "DOI": "10.1038/306234a0",
      "article-title": "A polymorphic DNA marker genetically linked to Huntington's disease",
      "volume": "306",
      "author": "Gusella",
      "year": "1983",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF68",
      "doi-asserted-by": "crossref",
      "first-page": "1157",
      "DOI": "10.1001/archneur.1993.00540110037003",
      "article-title": "Molecular genetics of Huntington's disease",
      "volume": "50",
      "author": "Gusella",
      "year": "1993",
      "journal-title": "Arch. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF69",
      "doi-asserted-by": "crossref",
      "first-page": "8710",
      "DOI": "10.1073/pnas.92.19.8710",
      "article-title": "Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein anti-bodies",
      "volume": "92",
      "author": "Gutekunst",
      "year": "1995",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF70",
      "doi-asserted-by": "crossref",
      "first-page": "233",
      "DOI": "10.1111/j.1399-0004.1998.tb02687.x",
      "article-title": "The fatal attraction of polyglutamine-containing proteins",
      "volume": "53",
      "author": "Hackam",
      "year": "1998",
      "journal-title": "Clin. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF71",
      "doi-asserted-by": "crossref",
      "first-page": "709",
      "DOI": "10.1016/0736-5748(93)90060-Q",
      "article-title": "Cellular transglutaminases in neural development",
      "volume": "11",
      "author": "Hand",
      "year": "1993",
      "journal-title": "Int. J. Dev. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF72",
      "doi-asserted-by": "crossref",
      "first-page": "365",
      "DOI": "10.1007/BF00194305",
      "article-title": "The epidemiology of Huntington's disease",
      "volume": "89",
      "author": "Harper",
      "year": "1992",
      "journal-title": "Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF73",
      "doi-asserted-by": "crossref",
      "first-page": "257",
      "DOI": "10.1016/0304-3940(91)90583-F",
      "article-title": "Neuronal loss in layer V and VI of cerebral cortex in Huntington's disease",
      "volume": "133",
      "author": "Hedreen",
      "year": "1991",
      "journal-title": "Neurosci. Lett."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF74",
      "doi-asserted-by": "crossref",
      "first-page": "105",
      "DOI": "10.1097/00005072-199501000-00013",
      "article-title": "Early loss of neostriatal striosome neurones in Huntington's disease",
      "volume": "54",
      "author": "Hedreen",
      "year": "1995",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF75",
      "first-page": "127.1",
      "article-title": "YAC transgenic mice expressing mutant human huntington show deficits in hippocampal long-term potentiation",
      "author": "Hodgson",
      "year": "1998",
      "journal-title": "Soc. Neurosci. Abstr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF76",
      "doi-asserted-by": "crossref",
      "first-page": "2069",
      "DOI": "10.1093/hmg/2.12.2069",
      "article-title": "Characterization and localization of the Huntington's disease gene product",
      "volume": "2",
      "author": "Hoogeveen",
      "year": "1993",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF77",
      "doi-asserted-by": "crossref",
      "first-page": "2811",
      "DOI": "10.1073/pnas.88.7.2811",
      "article-title": "Neuronal NADPH diaphorase is a nitric oxide synthase",
      "volume": "88",
      "author": "Hope",
      "year": "1991",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF78",
      "doi-asserted-by": "crossref",
      "first-page": "397",
      "DOI": "10.1016/0306-4522(94)00494-P",
      "article-title": "Quinolinic acid-induced increases in calbindin D28K immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease",
      "volume": "65",
      "author": "Huang",
      "year": "1995",
      "journal-title": "Neuroscience"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF79",
      "doi-asserted-by": "crossref",
      "first-page": "971",
      "DOI": "10.1016/0092-8674(93)90585-E",
      "volume": "72",
      "year": "1993",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF80",
      "doi-asserted-by": "crossref",
      "first-page": "2689",
      "DOI": "10.1212/WNL.43.12.2689",
      "article-title": "Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy",
      "volume": "43",
      "author": "Jenkins",
      "year": "1993",
      "journal-title": "Neurology"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF81",
      "doi-asserted-by": "crossref",
      "first-page": "125",
      "DOI": "10.1023/A:1005340302695",
      "article-title": "Huntington's disease: Advances in molecular and cell biology",
      "volume": "20",
      "author": "Jones",
      "year": "1997",
      "journal-title": "J. Inherit. Metab. Dis."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF82",
      "doi-asserted-by": "crossref",
      "first-page": "595",
      "DOI": "10.1016/S1097-2765(00)80059-3",
      "article-title": "Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine",
      "volume": "1",
      "author": "Kahlem",
      "year": "1998",
      "journal-title": "Mol. Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF83",
      "doi-asserted-by": "crossref",
      "first-page": "19385",
      "DOI": "10.1074/jbc.271.32.19385",
      "article-title": "Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme",
      "volume": "271",
      "author": "Kalchman",
      "year": "1996",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF84",
      "doi-asserted-by": "crossref",
      "first-page": "44",
      "DOI": "10.1038/ng0597-44",
      "article-title": "HIP1,S. cerevisiae Sla2p,",
      "volume": "16",
      "author": "Kalchman",
      "year": "1997",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF85",
      "doi-asserted-by": "crossref",
      "first-page": "239",
      "DOI": "10.1038/bjc.1972.33",
      "article-title": "Apoptosis: A basic biological phenomenon with wide ranging implications in tissue kinetics",
      "volume": "26",
      "author": "Kerr",
      "year": "1972",
      "journal-title": "Br. J. Cancer"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF86",
      "doi-asserted-by": "crossref",
      "first-page": "73",
      "DOI": "10.1126/science.2875522",
      "article-title": "Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity",
      "volume": "234",
      "author": "Koh",
      "year": "1986",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF87",
      "doi-asserted-by": "crossref",
      "first-page": "1311",
      "DOI": "10.1093/hmg/5.9.1311",
      "article-title": "Spinocerbellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase",
      "volume": "5",
      "author": "Koshy",
      "year": "1996",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF88",
      "doi-asserted-by": "crossref",
      "first-page": "24",
      "DOI": "10.1016/0166-2236(87)90120-2",
      "article-title": "Patterns of cell loss in Huntington's disease",
      "volume": "10",
      "author": "Kowall",
      "year": "1987",
      "journal-title": "Trends Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF89",
      "first-page": "735",
      "article-title": "Covariate-dependent age-at-onset distributions for Huntington's disease",
      "volume": "49",
      "author": "Krawczak",
      "year": "1991",
      "journal-title": "Am. J. Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF90",
      "doi-asserted-by": "crossref",
      "first-page": "1593",
      "DOI": "10.1212/WNL.43.8.1593",
      "article-title": "AMPA and NMDA binding sites in the hypothalamic lateral tuberal nucleus: Implications for Huntington's disease",
      "volume": "43",
      "author": "Kremer",
      "year": "1993",
      "journal-title": "Neurology"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF91",
      "doi-asserted-by": "crossref",
      "first-page": "371",
      "DOI": "10.1097/00005072-199007000-00002",
      "article-title": "Atrophy of the hypothalamic lateral tuberal nucleus in Huntington's Disease",
      "volume": "49",
      "author": "Kremer",
      "year": "1990",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF92",
      "doi-asserted-by": "crossref",
      "first-page": "349",
      "DOI": "10.1001/archneur.1992.00530280029014",
      "article-title": "Weight loss in Huntington's disease",
      "volume": "49",
      "author": "Kremer",
      "year": "1992",
      "journal-title": "Arch. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF93",
      "doi-asserted-by": "crossref",
      "first-page": "1405",
      "DOI": "10.1093/brain/113.5.1405",
      "article-title": "Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease",
      "volume": "113",
      "author": "Kuwert",
      "year": "1990",
      "journal-title": "Brain"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF94",
      "first-page": "380.8",
      "article-title": "Development and characterization of a novel transgenic model of Huntington's disease which recapitulates features of the human illness",
      "author": "Laforet",
      "year": "1998",
      "journal-title": "Soc. Neurosci. Abstr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF95",
      "doi-asserted-by": "crossref",
      "first-page": "183",
      "DOI": "10.1006/excr.1997.4026",
      "article-title": "Apoptosis, excitotoxicity, and neuropathology",
      "volume": "239",
      "author": "Leist",
      "year": "1998",
      "journal-title": "Exp. Cell Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF96",
      "first-page": "380.7",
      "article-title": "Enhanced sensitivity to glutamate receptor activation in mouse models of Huntington's disease",
      "author": "Levine",
      "year": "1998",
      "journal-title": "Soc. Neurosci. Abstr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF97",
      "doi-asserted-by": "crossref",
      "first-page": "777",
      "DOI": "10.1093/hmg/7.5.777",
      "article-title": "Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats",
      "volume": "7",
      "author": "Li",
      "year": "1998",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF98",
      "doi-asserted-by": "crossref",
      "first-page": "398",
      "DOI": "10.1038/378398a0",
      "article-title": "A huntingtin-associated protein enriched in brain with implications for pathology",
      "volume": "378",
      "author": "Li",
      "year": "1995",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF99",
      "doi-asserted-by": "crossref",
      "first-page": "4839",
      "DOI": "10.1073/pnas.93.10.4839",
      "article-title": "Huntingtin-associated protein (HAP1): Discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase",
      "volume": "93",
      "author": "Li",
      "year": "1996",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF100",
      "doi-asserted-by": "crossref",
      "first-page": "626",
      "DOI": "10.1038/364626a0",
      "article-title": "A redox-based mechanism for neuroprotective and neurodestructive effects of nitric oxide and related nitroso-compounds",
      "volume": "364",
      "author": "Lipton",
      "year": "1993",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF101",
      "doi-asserted-by": "crossref",
      "first-page": "613",
      "DOI": "10.1056/NEJM199403033300907",
      "article-title": "Excitatory amino acids as a final common pathway for neurological disorders",
      "volume": "330",
      "author": "Lipton",
      "year": "1994",
      "journal-title": "N. Engl. J. Med."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF102",
      "doi-asserted-by": "crossref",
      "first-page": "492",
      "DOI": "10.1017/S0317167100032212",
      "article-title": "3-Nitropropionic acid-exogenous animal toxin and possible human striatal toxin",
      "volume": "18",
      "author": "Ludolph",
      "year": "1991",
      "journal-title": "Can. J. Neurol. Sci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF103",
      "doi-asserted-by": "crossref",
      "first-page": "638",
      "DOI": "10.1016/S0959-4388(96)80097-3",
      "article-title": "Huntington's disease: Translating a CAG repeat into a pathogenetic model",
      "volume": "6",
      "author": "MacDonald",
      "year": "1996",
      "journal-title": "Curr. Opin. Neurobiol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF104",
      "series-title": "Huntington's Disease",
      "article-title": "The neurobiology of Huntington's disease",
      "author": "MacMillan",
      "year": "1996"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF105",
      "doi-asserted-by": "crossref",
      "first-page": "493",
      "DOI": "10.1016/S0092-8674(00)81369-0",
      "article-title": "Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice",
      "volume": "87",
      "author": "Mangiarini",
      "year": "1996",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF106",
      "doi-asserted-by": "crossref",
      "first-page": "197",
      "DOI": "10.1038/ng0297-197",
      "article-title": "Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation",
      "volume": "15",
      "author": "Mangiarini",
      "year": "1997",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF107",
      "doi-asserted-by": "crossref",
      "first-page": "653",
      "DOI": "10.1016/0896-6273(92)90087-T",
      "article-title": "Nitric oxide-induced blockade of NMDA receptors",
      "volume": "8",
      "author": "Manzoni",
      "year": "1992",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF108",
      "doi-asserted-by": "crossref",
      "first-page": "285",
      "DOI": "10.1046/j.1471-4159.1997.69010285.x",
      "article-title": "Iodoacetate produces striatal excitotoxic lesions",
      "volume": "69",
      "author": "Matthews",
      "year": "1997",
      "journal-title": "J. Neurochem."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF109",
      "doi-asserted-by": "crossref",
      "first-page": "381",
      "DOI": "10.1016/0006-8993(78)90941-1",
      "article-title": "Kainate-induced degeneration of neostriatal neurons: Dependency upon corticostriatal tract",
      "volume": "139",
      "author": "McGeer",
      "year": "1978",
      "journal-title": "Brain Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF110",
      "doi-asserted-by": "crossref",
      "first-page": "279",
      "DOI": "10.1016/S0306-4522(97)00594-0",
      "article-title": "Methylmalonate toxicity in primary neuronal cultures",
      "volume": "86",
      "author": "McLaughlin",
      "year": "1998",
      "journal-title": "Neuroscience"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF111",
      "doi-asserted-by": "crossref",
      "first-page": "775",
      "DOI": "10.1093/hmg/6.5.775",
      "article-title": "Reduced penetrance of the Huntington's disease mutation",
      "volume": "6",
      "author": "McNeil",
      "year": "1997",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF112",
      "first-page": "380.4",
      "article-title": "Conditional inactivation of huntingtin in transgenic mice",
      "author": "Metzler",
      "year": "1998",
      "journal-title": "Soc. Neurosci. Abstr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF113",
      "doi-asserted-by": "crossref",
      "first-page": "365",
      "DOI": "10.1146/annurev.pa.29.040189.002053",
      "article-title": "The excitatory amino acid receptors: Their classes, pharmacology, and distinct properties in the function of the central nervous system",
      "volume": "29",
      "author": "Monaghan",
      "year": "1989",
      "journal-title": "Annu. Rev. Pharmacol. Toxicol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF114",
      "doi-asserted-by": "crossref",
      "first-page": "513",
      "DOI": "10.1161/01.CIR.91.2.513",
      "article-title": "Unstable triplet repeat diseases",
      "volume": "91",
      "author": "Monckton",
      "year": "1995",
      "journal-title": "Circulation"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF115",
      "series-title": "Huntington's Disease",
      "first-page": "73",
      "article-title": "Psychiatric aspects of Huntington's disease",
      "author": "Morris",
      "year": "1996"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF116",
      "series-title": "Protection of Striatal and Nigral Neurons by Trophic and Antioxidant Mechanisms: A Study on in Vitro and in Vivo Brain Insult Models and Neural Transplants",
      "author": "Nakao",
      "year": "1996"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF117",
      "doi-asserted-by": "crossref",
      "first-page": "749",
      "DOI": "10.1016/S0306-4522(96)00223-0",
      "article-title": "Effects of alpha-phenyl-tert",
      "volume": "76",
      "author": "Nakao",
      "year": "1997",
      "journal-title": "Neuroscience"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF118",
      "doi-asserted-by": "crossref",
      "first-page": "811",
      "DOI": "10.1016/0092-8674(95)90542-1",
      "article-title": "Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes",
      "volume": "81",
      "author": "Nasir",
      "year": "1995",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF119",
      "doi-asserted-by": "crossref",
      "first-page": "299",
      "DOI": "10.1016/S0968-0004(97)01085-2",
      "article-title": "Caspases: Killer proteins",
      "volume": "22",
      "author": "Nicholson",
      "year": "1997",
      "journal-title": "Trends Biochem. Sci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF120",
      "doi-asserted-by": "crossref",
      "first-page": "139",
      "DOI": "10.1016/0304-3940(96)12794-4",
      "article-title": "In sity end-labeling detects necrosis of hippocampal pyramidal cells induced by kainic acid",
      "volume": "212",
      "author": "Nishiyama",
      "year": "1996",
      "journal-title": "Neurosci. Lett."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF121",
      "doi-asserted-by": "crossref",
      "first-page": "205",
      "DOI": "10.1016/0006-8993(88)90765-2",
      "article-title": "Glutamate becomes neurotoxic via theNd",
      "volume": "451",
      "author": "Novelli",
      "year": "1988",
      "journal-title": "Brain Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF122",
      "doi-asserted-by": "crossref",
      "first-page": "719",
      "DOI": "10.1126/science.164.3880.719",
      "article-title": "Brain lesion, obesity and other disturbances in mice treated with monosodium glutamate",
      "volume": "164",
      "author": "Olney",
      "year": "1969",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF123",
      "doi-asserted-by": "crossref",
      "first-page": "333",
      "DOI": "10.1016/S0896-6273(00)80943-5",
      "article-title": "Intranuclear inclusions of expanded polyglutamine protein in spinocerbellar ataxia type 3",
      "volume": "19",
      "author": "Paulson",
      "year": "1997",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF124",
      "doi-asserted-by": "crossref",
      "first-page": "3775",
      "DOI": "10.1523/JNEUROSCI.15-05-03775.1995",
      "article-title": "Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models",
      "volume": "15",
      "author": "Portera-Cailliau",
      "year": "1995",
      "journal-title": "J. Neurosci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF125",
      "doi-asserted-by": "crossref",
      "first-page": "5355",
      "DOI": "10.1073/pnas.91.12.5355",
      "article-title": "Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases",
      "volume": "91",
      "author": "Perutz",
      "year": "1994",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF126",
      "series-title": "Huntington's Disease",
      "first-page": "31",
      "article-title": "The clinical neurology of Huntington's disease",
      "author": "Quarrell",
      "year": "1996"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF127",
      "first-page": "593",
      "article-title": "Anticipation and instability of IT-15 (CAG)N",
      "volume": "57",
      "author": "Ranen",
      "year": "1995",
      "journal-title": "Am. J. Hum. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF128",
      "doi-asserted-by": "crossref",
      "first-page": "5733",
      "DOI": "10.1073/pnas.85.15.5733",
      "article-title": "Differential loss of striatal projection neurons in Huntington's disease",
      "volume": "85",
      "author": "Reiner",
      "year": "1988",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF129",
      "doi-asserted-by": "crossref",
      "first-page": "1256",
      "DOI": "10.1016/S0960-9822(02)70711-2",
      "article-title": "Rho: Theme and variations",
      "volume": "6",
      "author": "Ridley",
      "year": "1996",
      "journal-title": "Curr. Biol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF130",
      "doi-asserted-by": "crossref",
      "first-page": "1093",
      "DOI": "10.1083/jcb.130.5.1093",
      "article-title": "A mutation in glyceraldehyde-3-phosphate dehydrogenase alters endocytosis in CHO cells",
      "volume": "130",
      "author": "Robbins",
      "year": "1995",
      "journal-title": "J. Cell Biol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF131",
      "series-title": "Advances in Neurology, Vol. 23, Huntington's Disease",
      "first-page": "95",
      "article-title": "Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations",
      "author": "Roizin",
      "year": "1979"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF132",
      "first-page": "31",
      "article-title": "Nuclear membrane indentations in Huntington's chorea",
      "volume": "61",
      "author": "Ross",
      "year": "1983",
      "journal-title": "J. Neurol. Sci."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF133",
      "doi-asserted-by": "crossref",
      "first-page": "493",
      "DOI": "10.1016/0896-6273(95)90138-8",
      "article-title": "When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases",
      "volume": "15",
      "author": "Ross",
      "year": "1995",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF134",
      "doi-asserted-by": "crossref",
      "first-page": "1147",
      "DOI": "10.1016/S0896-6273(00)80405-5",
      "article-title": "Intranuclear neuronal inclusions: A common pathogenetic mechanism for glutamine-repeat neurodegenerative diseases",
      "volume": "19",
      "author": "Ross",
      "year": "1997",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF135",
      "doi-asserted-by": "crossref",
      "first-page": "407",
      "DOI": "10.5694/j.1326-5377.1981.tb135681.x",
      "article-title": "Body weight and dietary factors on Huntington's disease patients compared with matched controls",
      "volume": "1",
      "author": "Sanberg",
      "year": "1981",
      "journal-title": "Med. J. Aust."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF136",
      "doi-asserted-by": "crossref",
      "first-page": "604",
      "DOI": "10.1002/ana.410420411",
      "article-title": "Huntingtin localization in brains of normal and Huntington's disease patients",
      "volume": "42",
      "author": "Sapp",
      "year": "1997",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF137",
      "doi-asserted-by": "crossref",
      "first-page": "549",
      "DOI": "10.1016/S0092-8674(00)80514-0",
      "article-title": "Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo",
      "volume": "90",
      "author": "Scherzinger",
      "year": "1997",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF138",
      "doi-asserted-by": "crossref",
      "first-page": "261",
      "DOI": "10.1016/0197-4580(94)90128-7",
      "article-title": "Metabotropic glutamate receptors and neuronal degenerative disorders",
      "volume": "15",
      "author": "Schoepp",
      "year": "1994",
      "journal-title": "Neurobiol. Aging"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF139",
      "doi-asserted-by": "crossref",
      "first-page": "2239",
      "DOI": "10.1046/j.1471-4159.1995.64052239.x",
      "article-title": "Involvement of free radicals in excitotoxicityin vivo",
      "volume": "64",
      "author": "Schulz",
      "year": "1995",
      "journal-title": "J. Neurochem."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF140",
      "first-page": "110",
      "article-title": "Free radicals scavengers protect dopaminergic cell lines from apoptosis by complex I inhibitors",
      "volume": "777",
      "author": "Seaton",
      "year": "1997",
      "journal-title": "Brain Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF141",
      "doi-asserted-by": "crossref",
      "first-page": "1252",
      "DOI": "10.1016/S0140-6736(88)92073-9",
      "article-title": "Loss of matrix calcium-binding protein-containing neurons in Huntington's disease",
      "volume": "4",
      "author": "Seto-Oshima",
      "year": "1988",
      "journal-title": "Lancet"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF142",
      "doi-asserted-by": "crossref",
      "first-page": "1065",
      "DOI": "10.1016/0896-6273(95)90345-3",
      "article-title": "Widespread expression of Huntington's disease gene (IT15) protein product",
      "volume": "14",
      "author": "Sharp",
      "year": "1995",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF143",
      "doi-asserted-by": "crossref",
      "first-page": "3",
      "DOI": "10.1006/nbdi.1996.0002",
      "article-title": "Neurobiology of Huntington's disease",
      "volume": "3",
      "author": "Sharp",
      "year": "1996",
      "journal-title": "Neurobiol. Dis."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF144",
      "doi-asserted-by": "crossref",
      "first-page": "47",
      "DOI": "10.1006/nbdi.1998.0177",
      "article-title": "Neuronal death in cultured murine cortical cells is induced by inhibition of GAPDH and triosephosphate isomerase",
      "volume": "5",
      "author": "Sheline",
      "year": "1998",
      "journal-title": "Neurobiol. Dis."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF145",
      "doi-asserted-by": "crossref",
      "first-page": "7",
      "DOI": "10.1016/0014-5793(96)00989-1",
      "article-title": "Why are mitochondria involved in apoptosis? Permeability transition pores and apoptosis as selective mechanisms to eliminate superoxide-producing mitochondria and cell",
      "volume": "397",
      "author": "Skulachev",
      "year": "1996",
      "journal-title": "FEBS Lett."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF146",
      "doi-asserted-by": "crossref",
      "first-page": "393",
      "DOI": "10.1038/ng0893-393",
      "article-title": "Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease",
      "volume": "4",
      "author": "Snell",
      "year": "1993",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF147",
      "doi-asserted-by": "crossref",
      "first-page": "259",
      "DOI": "10.1038/ng1193-259",
      "article-title": "Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues",
      "volume": "5",
      "author": "Strong",
      "year": "1993",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF148",
      "doi-asserted-by": "crossref",
      "first-page": "1331",
      "DOI": "10.1084/jem.184.4.1331",
      "article-title": "Bcl-2 inhibits the mitochondrial release of an apoptogenic protease",
      "volume": "184",
      "author": "Susin",
      "year": "1996",
      "journal-title": "J. Exp. Med."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF149",
      "doi-asserted-by": "crossref",
      "first-page": "409",
      "DOI": "10.1038/ng0494-409",
      "article-title": "Somatic and gonadal mosaicism of the Huntington's disease gene CAG repeat in brain and sperm",
      "volume": "6",
      "author": "Telenius",
      "year": "1994",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF150",
      "doi-asserted-by": "crossref",
      "first-page": "308",
      "DOI": "10.1097/00005072-197404000-00008",
      "article-title": "Studies on brain biopsies of patients with Huntington's chorea",
      "volume": "33",
      "author": "Tellez-Nagel",
      "year": "1974",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF151",
      "doi-asserted-by": "crossref",
      "first-page": "265",
      "DOI": "10.1006/exnr.1995.1029",
      "article-title": "DNA end labeling (TUNEL) in Huntington's disease and other neuropathological conditions",
      "volume": "133",
      "author": "Thomas",
      "year": "1995",
      "journal-title": "Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF152",
      "doi-asserted-by": "crossref",
      "first-page": "1312",
      "DOI": "10.1126/science.281.5381.1312",
      "article-title": "Caspases: Enemies within",
      "volume": "281",
      "author": "Thornberry",
      "year": "1998",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF153",
      "doi-asserted-by": "crossref",
      "first-page": "377",
      "DOI": "10.1136/jmg.31.5.377",
      "article-title": "Instability of CAG repeats in Huntington's disease: Relation to parental transmission and age of onset",
      "volume": "31",
      "author": "Trottier",
      "year": "1994",
      "journal-title": "J. Med. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF154",
      "doi-asserted-by": "crossref",
      "first-page": "104",
      "DOI": "10.1038/ng0595-104",
      "article-title": "Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form",
      "volume": "10",
      "author": "Trottier",
      "year": "1995",
      "journal-title": "Nature Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF155",
      "doi-asserted-by": "crossref",
      "first-page": "8",
      "DOI": "10.1016/S0169-328X(97)00205-2",
      "article-title": "Huntington's disease gene product, huntingtin, associates with microtubules in vitro",
      "volume": "51",
      "author": "Tukamoto",
      "year": "1997",
      "journal-title": "Mol. Brain Res."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF156",
      "doi-asserted-by": "crossref",
      "first-page": "34",
      "DOI": "10.1006/exnr.1998.6832",
      "article-title": "Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways",
      "volume": "152",
      "author": "Velier",
      "year": "1998",
      "journal-title": "Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF157",
      "doi-asserted-by": "crossref",
      "first-page": "369",
      "DOI": "10.1097/00005072-199805000-00001",
      "article-title": "Huntington's disease",
      "volume": "57",
      "author": "Vonsattel",
      "year": "1998",
      "journal-title": "J. Neuropathol. Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF158",
      "doi-asserted-by": "crossref",
      "first-page": "758",
      "DOI": "10.1136/jnnp.64.6.758",
      "article-title": "Genetic polymorphisms adjacent to the CAG repeat influence clinical features at onset in Huntington's disease",
      "volume": "64",
      "author": "Vuillaume",
      "year": "1998",
      "journal-title": "J. Neurol. Neurosurg. Psychiatry"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF159",
      "doi-asserted-by": "crossref",
      "first-page": "70",
      "DOI": "10.1006/exnr.1994.1081",
      "article-title": "Selective loss of [33",
      "volume": "127",
      "author": "Wagster",
      "year": "1994",
      "journal-title": "Exp. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF160",
      "doi-asserted-by": "crossref",
      "first-page": "487",
      "DOI": "10.1093/hmg/6.3.487",
      "article-title": "HIP-1: A huntingtin interacting protein isolated by the yeast two-hybrid system",
      "volume": "6",
      "author": "Wanker",
      "year": "1997",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF161",
      "doi-asserted-by": "crossref",
      "first-page": "49",
      "DOI": "10.1002/ana.410400110",
      "article-title": "Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease",
      "volume": "40",
      "author": "Weeks",
      "year": "1996",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF162",
      "doi-asserted-by": "crossref",
      "first-page": "979",
      "DOI": "10.1111/j.1750-3639.1997.tb00897.x",
      "article-title": "Toward understanding the molecular pathology of Huntington's disease",
      "volume": "7",
      "author": "Wellington",
      "year": "1997",
      "journal-title": "Brain Pathol."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF163",
      "doi-asserted-by": "crossref",
      "first-page": "194",
      "DOI": "10.1038/326194a0",
      "article-title": "Homozygotes for Huntington's disease",
      "volume": "326",
      "author": "Wexler",
      "year": "1987",
      "journal-title": "Nature"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF164",
      "series-title": "Cell Death in Biology and Pathology",
      "first-page": "9",
      "article-title": "Cell death: A new classification separating apoptosis from necrosis",
      "author": "Wyllie",
      "year": "1981"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF165",
      "doi-asserted-by": "crossref",
      "first-page": "981",
      "DOI": "10.1126/science.2841762",
      "article-title": "NMDA receptor losses in putamen from patients with Huntington's disease",
      "volume": "241",
      "author": "Young",
      "year": "1988",
      "journal-title": "Science"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF166",
      "doi-asserted-by": "crossref",
      "first-page": "641",
      "DOI": "10.1016/0092-8674(93)90485-9",
      "article-title": "TheC. elegans",
      "volume": "75",
      "author": "Yuan",
      "year": "1993",
      "journal-title": "Cell"
    },
    {
      "key": "10.1006/exnr.1998.7006_RF167",
      "doi-asserted-by": "crossref",
      "first-page": "185",
      "DOI": "10.1023/A:1022694131572",
      "article-title": "Mitochondrial implication in accidental and programmed cell death: Apoptosis and necrosis",
      "volume": "29",
      "author": "Zamzami",
      "year": "1997",
      "journal-title": "J. Bionerg. Biomembr."
    },
    {
      "key": "10.1006/exnr.1998.7006_RF168",
      "doi-asserted-by": "crossref",
      "first-page": "155",
      "DOI": "10.1038/ng1095-155",
      "article-title": "Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue",
      "volume": "11",
      "author": "Zeitlin",
      "year": "1995",
      "journal-title": "Nature Genet."
    }
  ],
  "container-title": "Experimental Neurology",
  "original-title": [],
  "language": "en",
  "link": [
    {
      "URL": "https://api.elsevier.com/content/article/PII:S0014488698970066?httpAccept=text/xml",
      "content-type": "text/xml",
      "content-version": "vor",
      "intended-application": "text-mining"
    },
    {
      "URL": "https://api.elsevier.com/content/article/PII:S0014488698970066?httpAccept=text/plain",
      "content-type": "text/plain",
      "content-version": "vor",
      "intended-application": "text-mining"
    }
  ],
  "deposited": {
    "date-parts": [
      [
        2019,
        12,
        17
      ]
    ],
    "date-time": "2019-12-17T13:24:15Z",
    "timestamp": 1576589055000
  },
  "score": 1,
  "resource": {
    "primary": {
      "URL": "https://linkinghub.elsevier.com/retrieve/pii/S0014488698970066"
    }
  },
  "subtitle": [],
  "short-title": [],
  "issued": {
    "date-parts": [
      [
        1999,
        5
      ]
    ]
  },
  "references-count": 168,
  "journal-issue": {
    "issue": "1",
    "published-print": {
      "date-parts": [
        [
          1999,
          5
        ]
      ]
    }
  },
  "alternative-id": [
    "S0014488698970066"
  ],
  "URL": "http://dx.doi.org/10.1006/exnr.1998.7006",
  "relation": {},
  "ISSN": [
    "0014-4886"
  ],
  "subject": [],
  "container-title-short": "Experimental Neurology",
  "published": {
    "date-parts": [
      [
        1999,
        5
      ]
    ]
  }
}