Bibliography
Rockabrand, E., Slepko, N., Pantalone, A., Nukala, V. N., Kazantsev, A., Marsh, J. L., Sullivan, P. G., Steffan, J. S., Sensi, S. L., & Thompson, L. M. (2006). The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. Human Molecular Genetics, 16(1), 61â77.
Authors
10
- Erica Rockabrand (first)
- Natalia Slepko (additional)
- Antonello Pantalone (additional)
- Vidya N. Nukala (additional)
- Aleksey Kazantsev (additional)
- J. Lawrence Marsh (additional)
- Patrick G. Sullivan (additional)
- Joan S. Steffan (additional)
- Stefano L. Sensi (additional)
- Leslie Michels Thompson (additional)
References
95
Referenced
231
10.1038/nrn1806
/ Nat. Rev. Neurosci. / Normal huntingtin function: an alternative approach to Huntington's disease by Cattaneo (2005)10.1101/gad.1436506
/ Genes Dev. / Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle by Riley (2006)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)10.1126/science.277.5334.1990
/ Science / Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain by DiFiglia (1997)10.1016/j.nbd.2005.07.014
/ Neurobiol. Dis. / Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin by Tanaka (2006)10.1016/j.cell.2006.04.026
/ Cell / Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin by Graham (2006)10.1074/jbc.M106575200
/ J. Biol. Chem. / The role of protein composition in specifying nuclear inclusion formation in polyglutamine disease by Chai (2001)10.1097/00001756-200110290-00042
/ Neuroreport / Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation by Nozaki (2001)10.1073/pnas.022631799
/ Proc. Natl Acad. Sci. USA / Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity by Khoshnan (2002)10.1073/pnas.071058398
/ Proc. Natl Acad. Sci. USA / Human single-chain Fv intrabodies counteract in situ huntingtin aggregation in cellular models of Huntington's Disease by Lecerf (2001)10.1126/science.1092194
/ Science / SUMO modification of Huntingtin and Huntington's disease pathology by Steffan (2004)10.1093/hmg/ddi340
/ Hum. Mol. Genet. / Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease by Benn (2005)10.1006/mcne.1999.0773
/ Mol. Cell Neurosci. / Nuclear targeting of mutant Huntingtin increases toxicity by Peters (1999)10.1016/S0092-8674(00)81782-1
/ Cell / Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions by Saudou (1998)10.1093/hmg/ddh175
/ Hum. Mol. Genet. / Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice by Schilling (2004)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)10.1523/JNEUROSCI.1409-03.2004
/ J. Neurosci. / Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction by Qin (2004)10.1016/j.cell.2004.06.018
/ Cell / Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules by Gauthier (2004)10.1073/pnas.2034961100
/ Proc. Natl Acad. Sci. USA / Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease by Trushina (2003)10.1016/S0896-6273(00)80764-3
/ Neuron / A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration by Hodgson (1999)10.1002/(SICI)1097-4547(19991115)58:4<515::AID-JNR5>3.0.CO;2-F
/ J. Neurosci. Res. / Enhanced sensitivity to N-methyl- d -aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease by Levine (1999)10.1016/S0896-6273(02)00615-3
/ Neuron / Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease by Zeron (2002)10.1016/j.mcn.2003.11.014
/ Mol. Cell Neurosci. / Potentiation of NMDA receptor-mediated excitotoxicity linked with intrinsic apoptotic pathway in YAC transgenic mouse model of Huntington's disease by Zeron (2004)10.1093/hmg/9.19.2789
/ Hum. Mol. Genet. / Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles by Hilditch-Maguire (2000)10.1073/pnas.0409402102
/ Proc. Natl Acad. Sci. USA / Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease by Tang (2005)10.1016/S0896-6273(03)00366-0
/ Neuron / Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1 by Tang (2003)10.1016/j.bbrc.2004.08.035
/ Biochem. Biophys. Res. Commun. / Deranged neuronal calcium signaling and Huntington disease by Bezprozvanny (2004)10.1074/jbc.M603845200
/ J. Biol. Chem / Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: Functional consequences by Milakovic (2006)10.1038/nn884
/ Nat. Neurosci. / Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines by Panov (2002)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)10.1523/JNEUROSCI.13-10-04181.1993
/ J. Neurosci. / Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid by Beal (1993)10.1093/hmg/ddh162
/ Hum. Mol. Genet. / Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release by Choo (2004)10.1523/JNEUROSCI.18-19-07674.1998
/ J. Neurosci. / The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human by Gutekunst (1998)10.1523/JNEUROSCI.23-06-02193.2003
/ J. Neurosci. / Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease by Yu (2003)10.1074/jbc.M504749200
/ J. Biol. Chem. / Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin by Milakovic (2005)10.1007/s11010-005-3454-9
/ Mol. Cell Biochem. / Ca2+-induced permeability transition in human lymphoblastoid cell mitochondria from normal and Huntington's disease individuals by Panov (2005)10.1073/pnas.0501651102
/ Proc. Natl Acad. Sci. USA / polyglutamine aggregation nucleation: thermodynamics of a highly unfavorable protein folding reaction by Bhattacharyya (2005)10.1073/pnas.2628045100
/ Proc. Natl Acad. Sci. USA / A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila by Apostol (2003)10.1016/S0003-9861(02)00585-4
/ Arch. Biochem. Biophys. / In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease by Panov (2003)10.1016/j.bbrc.2006.01.007
/ Biochem. Biophys. Res. Commun. / Polyglutamine expansion inhibits respiration by increasing reactive oxygen species in isolated mitochondria by Puranam (2006)10.1146/annurev.biochem.66.1.863
/ Annu. Rev. Biochem. / Protein import into mitochondria by Neupert (1997)10.1016/0003-9861(92)90370-C
/ Arch. Biochem. Biophys. / Expression and function of heterologous forms of malate dehydrogenase in yeast by Steffan (1992)10.1093/hmg/9.19.2799
/ Hum. Mol. Genet. / Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells by Trettel (2000)10.1074/jbc.M503672200
/ J. Biol. Chem. / Huntingtin associates with acidic phospholipids at the plasma membrane by Kegel (2005)10.1083/jcb.200508072
/ J. Cell Biol. / Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity by Shin (2005)10.1016/j.nbd.2005.12.007
/ Neurobiol. Dis. / Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons by Chang (2006)10.1523/JNEUROSCI.21-21-08473.2001
/ J. Neurosci. / Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice by Li (2001)10.1016/S1097-2765(02)00602-0
/ Mol. Cell / Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions by Lunkes (2002)10.1523/JNEUROSCI.22-09-03473.2002
/ J. Neurosci. / Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length by de Almeida (2002)10.1073/pnas.96.13.7388
/ Proc. Natl Acad. Sci. USA / Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei by Karpuj (1999)10.1073/pnas.0403015101
/ Proc. Natl Acad. Sci. USA / Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation by Rousseau (2004)10.1016/0024-3205(94)90012-4
/ Life Sci. / Trinucleotide repeats in neurologic diseases: an hypothesis concerning the pathogenesis of Huntington's disease, Kennedy's disease, and spinocerebellar ataxia type I by Cha (1994)10.1093/hmg/ddl013
/ Hum. Mol. Genet. / Regional and cellular gene expression changes in human Huntington's disease brain by Hodges (2006)10.1074/jbc.M103501200
/ J. Biol. Chem. / Polyglutamine-expanded huntingtin promotes sensitization of N -methyl- d -aspartate receptors via post-synaptic density 95 by Sun (2001)10.1111/j.1471-4159.2005.03515.x
/ J. Neurochem. / 3-Nitropropionic acid: a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease by Brouillet (2005)10.1038/13518
/ Nat. Med. / Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization by Sawa (1999)10.1093/hmg/ddi319
/ Hum. Mol. Genet. / HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism by Seong (2005)10.1074/jbc.M500997200
/ J. Biol. Chem. / Calcium dysregulation and membrane disruption as a ubiquitous neurotoxic mechanism of soluble amyloid oligomers by Demuro (2005)10.1002/ana.10543
/ Ann. Neurol. / Mitochondrial uncoupling protein-2 protects the immature brain from excitotoxic neuronal death by Sullivan (2003)10.1002/ana.20062
/ Ann. Neurol. / The ketogenic diet increases mitochondrial uncoupling protein levels and activity by Sullivan (2004)10.1089/ars.2006.8.1
/ Antioxid. Redox Signal / The emerging functions of UCP2 in health, disease, and therapeutics by Mattiasson (2006)10.1016/j.nbd.2005.08.001
/ Neurobiol. Dis. / Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease by Shehadeh (2006)10.1016/S0166-2236(00)01609-X
/ Trends Neurosci. / Transcriptional dysregulation in Huntington's disease by Cha (2000)10.1093/hmg/11.17.1911
/ Hum. Mol. Genet. / Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain by Luthi-Carter (2002)10.1073/pnas.0437870100
/ Proc. Natl Acad. Sci. USA / Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease by Hockly (2003)10.1038/35099568
/ Nature / Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila by Steffan (2001)10.1007/s00702-004-0201-4
/ J. Neural Transm. / Huntington's disease: pathomechanism and therapeutic perspectives by Gardian (2004)10.1073/pnas.0510477103
/ Proc. Natl. Acad. Sci. USA / Elk-1 associates with the mitochondrial permeability transition pore complex in neurons by Barrett (2006)10.1074/jbc.M005850200
/ J. Biol. Chem. / Ikappa b-alpha, the NF-kappa B inhibitory subunit, interacts with ANT, the mitochondrial ATP/ADP translocator by Bottero (2001)10.1126/science.1092734
/ Science / Direct activation of Bax by p53 mediates mitochondrial membrane permeabilization and apoptosis by Chipuk (2004)10.1074/jbc.275.21.16202
/ J. Biol. Chem. / Death signal-induced localization of p53 protein to mitochondria. A potential role in apoptotic signaling by Marchenko (2000)10.1101/gad.1114503
/ Genes Dev. / NF-kappaB mediates inhibition of mesenchymal cell differentiation through a posttranscriptional gene silencing mechanism by Sitcheran (2003)10.1074/jbc.M404928200
/ J. Biol.Chem. / Recruitment of NF-kappaB into mitochondria is involved in adenine nucleotide translocase 1 (ANT1)-induced apoptosis by Zamora (2004)10.1016/j.cell.2006.09.015
/ Cell / Transcriptional Repression of PGC-1alpha by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration by Cui (2006)10.1016/j.cmet.2006.10.004
/ Cell Metab. / Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC1alpha in Huntington's disease neurodegeneration by Weydt (2006)10.4161/cbt.190
/ Cancer Biol. Ther. / Histone deacetylase inhibitors in cancer therapy by Rosato (2003)10.1523/JNEUROSCI.3004-06.2006
/ J. Neurosci. / Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors by Oliveira (2006)10.1016/j.bbrc.2005.10.061
/ Biochem. Biophys. Res. Commun. / siRNA-mediated inhibition of endogenous Huntington disease gene expression induces an aberrant configuration of the ER network in vitro by Omi (2005)10.1111/j.1471-4159.2005.03620.x
/ J. Neurochem. / Interaction of huntingtin fragments with brain membranes–clues to early dysfunction in Huntington's disease by Suopanki (2006)10.1128/MCB.24.18.8195-8209.2004
/ Mol. Cell. Biol. / Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro by Trushina (2004)10.1073/pnas.0604547103
/ Proc. Natl Acad. Sci. USA / Flanking sequences profoundly alter polyglutamine toxicity in yeast by Duennwald (2006)10.1016/j.jmb.2005.10.053
/ J. Mol. Biol. / Oligoproline Effects on Polyglutamine Conformation and Aggregation by Bhattacharyya (2006)10.1038/nature02998
/ Nature / Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death by Arrasate (2004)10.1038/nrm1742
/ Nat. Rev. Mol. Cell Biol. / Opinion: What is the role of protein aggregation in neurodegeneration? by Ross (2005)10.1016/j.mad.2006.01.022
/ Mech. Ageing Dev. / Aging and neurodegeneration Molecular mechanisms of neuronal loss in Huntington's disease by Lee (2006)10.1212/01.wnl.0000192103.24796.42
/ Neurology / Common structure and toxic function of amyloid oligomers implies a common mechanism of pathogenesis by Glabe (2006)10.1073/pnas.100110097
/ Proc. Natl Acad. Sci. USA / The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription by Steffan (2000)10.1073/pnas.96.20.11404
/ Proc. Natl Acad. Sci. USA / Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells by Kazantsev (1999)10.1093/hmg/11.17.1953
/ Hum. Mol. Genet. / Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses by Sipione (2002)10.1111/j.1365-2818.1993.tb03313.x
/ J. Microsc. / Measurement of co-localization of objects in dual-colour confocal images by Manders (1993)10.1002/1531-8249(200011)48:5<723::AID-ANA5>3.0.CO;2-W
/ Ann. Neurol. / Dietary supplement creatine protects against traumatic brain injury by Sullivan (2000)10.1023/B:NERE.0000014824.04728.dd
/ Neurochem. Res. / The energetics of Huntington's disease by Browne (2004)10.1016/S1385-299X(99)00060-4
/ Brain Res. Brain Res. Protoc. / Improved recovery of highly enriched mitochondrial fractions from small brain tissue samples by Anderson (2000)10.1073/pnas.1031598100
/ Proc. Natl Acad. Sci. USA / Modulation of mitochondrial function by endogenous Zn2+ pools by Sensi (2003)10.1006/exnr.1999.7197
/ Exp. Neurol. / Cyclosporin A attenuates acute mitochondrial dysfunction following traumatic brain injury by Sullivan (1999)
Dates
Type | When |
---|---|
Created | 18 years, 8 months ago (Nov. 29, 2006, 8:25 p.m.) |
Deposited | 6 years, 4 months ago (April 22, 2019, 1:04 p.m.) |
Indexed | 3 days, 3 hours ago (Aug. 26, 2025, 2:59 a.m.) |
Issued | 18 years, 9 months ago (Nov. 29, 2006) |
Published | 18 years, 9 months ago (Nov. 29, 2006) |
Published Online | 18 years, 9 months ago (Nov. 29, 2006) |
Published Print | 18 years, 7 months ago (Jan. 1, 2007) |
@article{Rockabrand_2006, title={The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis}, volume={16}, ISSN={0964-6906}, url={http://dx.doi.org/10.1093/hmg/ddl440}, DOI={10.1093/hmg/ddl440}, number={1}, journal={Human Molecular Genetics}, publisher={Oxford University Press (OUP)}, author={Rockabrand, Erica and Slepko, Natalia and Pantalone, Antonello and Nukala, Vidya N. and Kazantsev, Aleksey and Marsh, J. Lawrence and Sullivan, Patrick G. and Steffan, Joan S. and Sensi, Stefano L. and Thompson, Leslie Michels}, year={2006}, month=nov, pages={61–77} }