DOI: 10.1016/j.molcel.2004.12.021. Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation

Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation

10.1016/j.molcel.2004.12.021

Crossref journal-article

Elsevier BV

Molecular Cell (78)

Bibliography

Bennett, E. J., Bence, N. F., Jayakumar, R., & Kopito, R. R. (2005). Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation. Molecular Cell, 17(3), 351â365.

Authors 4

Eric J. Bennett (first)
Neil F. Bence (additional)
Rajadas Jayakumar (additional)
Ron R. Kopito (additional)

References 56 Referenced 415

10.1074/jbc.272.3.1791 / J. Biol. Chem. / Processive degradation of proteins and other catalytic properties of the proteasome from Thermoplasma acidophilum by Akopian (1997)
10.1038/nature02998 / Nature / Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death by Arrasate (2004)
10.1074/jbc.272.41.25560 / J. Biol. Chem. / Molecular cloning of a novel ubiquitin-specific protease, UBP41, with isopeptidase activity in chick skeletal muscle by Baek (1997)
10.1126/science.292.5521.1552 / Science / Impairment of the ubiquitin-proteasome system by protein aggregation by Bence (2001)
10.1038/416507a / Nature / Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases by Bucciantini (2002)
10.1101/gad.898701 / Genes Dev. / MAD2B is an inhibitor of the anaphase-promoting complex by Chen (2001)
10.1110/ps.42301 / Protein Sci. / Solubilization and disaggregation of polyglutamine peptides by Chen (2001)
10.1006/jmbi.2001.4850 / J. Mol. Biol. / Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity by Chen (2001)
10.1038/35056508 / Nat. Rev. Mol. Cell Biol. / Regulation of cellular polyamines by antizyme by Coffino (2001)
10.1038/502 / Nat. Genet. / Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 by Cummings (1998)
10.1098/rstb.1999.0462 / Philos. Trans. R. Soc. Lond. B Biol. Sci. / Progress in pathogenesis studies of spinocerebellar ataxia type 1 by Cummings (1999)
10.1016/S0896-6273(00)81035-1 / Neuron / Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice by Cummings (1999)
10.1073/pnas.1530212100 / Proc. Natl. Acad. Sci. USA / Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates by Donaldson (2003)
10.1038/35040584 / Nature / Identification of genes that modify ataxin-1-induced neurodegeneration by Fernandez-Funez (2000)
10.1016/S1359-0278(98)00002-9 / Fold. Des. / Protein aggregation by Fink (1998)
10.1016/0092-8674(95)90436-0 / Cell / The HIV-1 Rev activation domain is a nuclear export signal that accesses an export pathway used by specific cellular RNAs by Fischer (1995)
10.1016/S0896-6273(03)00594-4 / Neuron / Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila by Gunawardena (2003)
10.1093/hmg/8.1.25 / Hum. Mol. Genet. / In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease by Hackam (1999)
10.1126/science.1079694 / Science / Spongiform degeneration in mahoganoid mutant mice by He (2003)
10.1146/annurev.biochem.67.1.425 / Annu. Rev. Biochem. / The ubiquitin system by Hershko (1998)
10.1038/sj.emboj.7600426 / EMBO J. / Inefficient degradation of truncated polyglutamine proteins by the proteasome by Holmberg (2004)
10.1074/jbc.M204955200 / J. Biol. Chem. / A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system by Illing (2002)
10.1093/hmg/10.10.1049 / Hum. Mol. Genet. / Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release by Jana (2001)
10.1073/pnas.220417997 / Proc. Natl. Acad. Sci. USA / Formation of high molecular weight complexes of mutant cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis by Johnston (2000)
10.1038/311033a0 / Nature / Sequence requirements for nuclear location of simian virus 40 large-T antigen by Kalderon (1984)
10.1016/S0092-8674(03)00939-5 / Cell / The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress by Kawaguchi (2003)
10.1126/science.1079469 / Science / Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis by Kayed (2003)
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)
10.1038/ncb863 / Nat. Cell Biol. / Polyglutamine protein aggregates are dynamic by Kim (2002)
10.1016/S0092-8674(00)81781-X / Cell / Ataxin-1 nuclear localization and aggregation by Klement (1998)
10.1073/pnas.96.18.9989 / Proc. Natl. Acad. Sci. USA / Amyloid diseases by Koo (1999)
10.1016/S0962-8924(00)01852-3 / Trends Cell Biol. / Aggresomes, inclusion bodies and protein aggregation by Kopito (2000)
10.1038/418291a / Nature / Neurodegenerative disease by Lashuel (2002)
10.1097/00019052-200108000-00008 / Curr. Opin. Neurol. / Parkin and Parkinson's disease by Mizuno (2001)
10.1126/science.1056784 / Science / Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity by Nucifora (2001)
10.1073/pnas.91.12.5355 / Proc. Natl. Acad. Sci. USA / Glutamine repeats as polar zippers by Perutz (1994)
10.1006/mcne.1999.0773 / Mol. Cell. Neurosci. / Nuclear targeting of mutant Huntingtin increases toxicity by Peters (1999)
10.1074/jbc.M205809200 / J. Biol. Chem. / Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization by Poirier (2002)
10.1093/emboj/16.20.6087 / EMBO J. / Dynamics of proteasome distribution in living cells by Reits (1997)
10.1038/12647 / Nat. Genet. / Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice by Saigoh (1999)
10.1242/jcs.115.14.2907 / J. Cell Sci. / The cellular fate of mutant rhodopsin by Saliba (2002)
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)
10.1016/j.molcel.2004.06.029 / Mol. Cell / Cellular toxicity of polyglutamine expansion proteins; mechanism of transcription factor deactivation by Schaffar (2004)
10.1073/pnas.96.8.4604 / Proc. Natl. Acad. Sci. USA / Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils by Scherzinger (1999)
10.1002/ana.10101 / Ann. Neurol. / Protein surveillance machinery in brains with spinocerebellar ataxia type 3 by Schmidt (2002)
10.1038/ncb859 / Nat. Cell Biol. / Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components by Stenoien (2002)
10.1016/S1097-2765(04)00156-X / Mol. Cell / Caspase activation inhibits proteasome function during apoptosis by Sun (2004)
10.1016/S0021-9258(19)74360-9 / J. Biol. Chem. / Rhodopsin mutations responsible for autosomal dominant retinitis pigmentosa. Clustering of functional classes along the polypeptide chain by Sung (1993)
10.1126/science.1067122 / Science / Toxic proteins in neurodegenerative disease by Taylor (2002)
10.1093/hmg/ddg074 / Hum. Mol. Genet. / Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein by Taylor (2003)
10.1093/emboj/cdg044 / EMBO J. / From Alzheimer to Huntington by Temussi (2003)
10.1016/S0166-2236(99)01409-5 / Trends Neurosci. / Aggregates in neurodegenerative disease by Tran (1999)
10.1016/S1097-2765(04)00151-0 / Mol. Cell / Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins by Venkatraman (2004)
10.1093/hmg/11.22.2689 / Hum. Mol. Genet. / Aggregate formation inhibits proteasomal degradation of polyglutamine proteins by Verhoef (2002)
10.1515/BC.2000.114 / Biol. Chem. / Protein aggregation and pathogenesis of Huntington's disease by Wanker (2000)
10.1016/S0076-6879(99)09026-6 / Methods Enzymol. / Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates by Wanker (1999)

Dates

Type	When
Created	20 years, 6 months ago (Feb. 4, 2005, 7:12 a.m.)
Deposited	4 years, 1 month ago (July 4, 2021, 12:56 p.m.)
Indexed	3 weeks, 6 days ago (July 26, 2025, 5:14 a.m.)
Issued	20 years, 6 months ago (Feb. 1, 2005)
Published	20 years, 6 months ago (Feb. 1, 2005)
Published Print	20 years, 6 months ago (Feb. 1, 2005)

Funders 0

None

BibTeX

@article{Bennett_2005, title={Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation}, volume={17}, ISSN={1097-2765}, url={http://dx.doi.org/10.1016/j.molcel.2004.12.021}, DOI={10.1016/j.molcel.2004.12.021}, number={3}, journal={Molecular Cell}, publisher={Elsevier BV}, author={Bennett, Eric J. and Bence, Neil F. and Jayakumar, Rajadas and Kopito, Ron R.}, year={2005}, month=feb, pages={351–365} }

JSON

{
  "indexed": {
    "date-parts": [
      [
        2025,
        7,
        26
      ]
    ],
    "date-time": "2025-07-26T09:14:24Z",
    "timestamp": 1753521264349
  },
  "reference-count": 56,
  "publisher": "Elsevier BV",
  "issue": "3",
  "license": [
    {
      "start": {
        "date-parts": [
          [
            2005,
            2,
            1
          ]
        ],
        "date-time": "2005-02-01T00:00:00Z",
        "timestamp": 1107216000000
      },
      "content-version": "tdm",
      "delay-in-days": 0,
      "URL": "https://www.elsevier.com/tdm/userlicense/1.0/"
    },
    {
      "start": {
        "date-parts": [
          [
            2013,
            7,
            17
          ]
        ],
        "date-time": "2013-07-17T00:00:00Z",
        "timestamp": 1374019200000
      },
      "content-version": "vor",
      "delay-in-days": 3088,
      "URL": "https://www.elsevier.com/open-access/userlicense/1.0/"
    }
  ],
  "content-domain": {
    "domain": [],
    "crossmark-restriction": false
  },
  "published-print": {
    "date-parts": [
      [
        2005,
        2
      ]
    ]
  },
  "DOI": "10.1016/j.molcel.2004.12.021",
  "type": "journal-article",
  "created": {
    "date-parts": [
      [
        2005,
        2,
        4
      ]
    ],
    "date-time": "2005-02-04T12:12:48Z",
    "timestamp": 1107519168000
  },
  "page": "351-365",
  "source": "Crossref",
  "is-referenced-by-count": 415,
  "title": "Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation",
  "prefix": "10.1016",
  "volume": "17",
  "author": [
    {
      "given": "Eric J.",
      "family": "Bennett",
      "sequence": "first",
      "affiliation": []
    },
    {
      "given": "Neil F.",
      "family": "Bence",
      "sequence": "additional",
      "affiliation": []
    },
    {
      "given": "Rajadas",
      "family": "Jayakumar",
      "sequence": "additional",
      "affiliation": []
    },
    {
      "given": "Ron R.",
      "family": "Kopito",
      "sequence": "additional",
      "affiliation": []
    }
  ],
  "member": "78",
  "reference": [
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB1",
      "doi-asserted-by": "crossref",
      "first-page": "1791",
      "DOI": "10.1074/jbc.272.3.1791",
      "article-title": "Processive degradation of proteins and other catalytic properties of the proteasome from Thermoplasma acidophilum",
      "volume": "272",
      "author": "Akopian",
      "year": "1997",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB2",
      "doi-asserted-by": "crossref",
      "first-page": "805",
      "DOI": "10.1038/nature02998",
      "article-title": "Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death",
      "volume": "431",
      "author": "Arrasate",
      "year": "2004",
      "journal-title": "Nature"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB3",
      "doi-asserted-by": "crossref",
      "first-page": "25560",
      "DOI": "10.1074/jbc.272.41.25560",
      "article-title": "Molecular cloning of a novel ubiquitin-specific protease, UBP41, with isopeptidase activity in chick skeletal muscle",
      "volume": "272",
      "author": "Baek",
      "year": "1997",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB4",
      "doi-asserted-by": "crossref",
      "first-page": "1552",
      "DOI": "10.1126/science.292.5521.1552",
      "article-title": "Impairment of the ubiquitin-proteasome system by protein aggregation",
      "volume": "292",
      "author": "Bence",
      "year": "2001",
      "journal-title": "Science"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB5",
      "doi-asserted-by": "crossref",
      "first-page": "507",
      "DOI": "10.1038/416507a",
      "article-title": "Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases",
      "volume": "416",
      "author": "Bucciantini",
      "year": "2002",
      "journal-title": "Nature"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB6",
      "doi-asserted-by": "crossref",
      "first-page": "1765",
      "DOI": "10.1101/gad.898701",
      "article-title": "MAD2B is an inhibitor of the anaphase-promoting complex",
      "volume": "15",
      "author": "Chen",
      "year": "2001",
      "journal-title": "Genes Dev."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB7",
      "doi-asserted-by": "crossref",
      "first-page": "887",
      "DOI": "10.1110/ps.42301",
      "article-title": "Solubilization and disaggregation of polyglutamine peptides",
      "volume": "10",
      "author": "Chen",
      "year": "2001",
      "journal-title": "Protein Sci."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB8",
      "doi-asserted-by": "crossref",
      "first-page": "173",
      "DOI": "10.1006/jmbi.2001.4850",
      "article-title": "Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity",
      "volume": "311",
      "author": "Chen",
      "year": "2001",
      "journal-title": "J. Mol. Biol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB9",
      "doi-asserted-by": "crossref",
      "first-page": "188",
      "DOI": "10.1038/35056508",
      "article-title": "Regulation of cellular polyamines by antizyme",
      "volume": "2",
      "author": "Coffino",
      "year": "2001",
      "journal-title": "Nat. Rev. Mol. Cell Biol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB10",
      "doi-asserted-by": "crossref",
      "first-page": "148",
      "DOI": "10.1038/502",
      "article-title": "Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1",
      "volume": "19",
      "author": "Cummings",
      "year": "1998",
      "journal-title": "Nat. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB11",
      "doi-asserted-by": "crossref",
      "first-page": "1079",
      "DOI": "10.1098/rstb.1999.0462",
      "article-title": "Progress in pathogenesis studies of spinocerebellar ataxia type 1",
      "volume": "354",
      "author": "Cummings",
      "year": "1999",
      "journal-title": "Philos. Trans. R. Soc. Lond. B Biol. Sci."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB12",
      "doi-asserted-by": "crossref",
      "first-page": "879",
      "DOI": "10.1016/S0896-6273(00)81035-1",
      "article-title": "Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice",
      "volume": "24",
      "author": "Cummings",
      "year": "1999",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB13",
      "doi-asserted-by": "crossref",
      "first-page": "8892",
      "DOI": "10.1073/pnas.1530212100",
      "article-title": "Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates",
      "volume": "100",
      "author": "Donaldson",
      "year": "2003",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB14",
      "doi-asserted-by": "crossref",
      "first-page": "101",
      "DOI": "10.1038/35040584",
      "article-title": "Identification of genes that modify ataxin-1-induced neurodegeneration",
      "volume": "408",
      "author": "Fernandez-Funez",
      "year": "2000",
      "journal-title": "Nature"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB15",
      "doi-asserted-by": "crossref",
      "first-page": "R9",
      "DOI": "10.1016/S1359-0278(98)00002-9",
      "article-title": "Protein aggregation",
      "volume": "3",
      "author": "Fink",
      "year": "1998",
      "journal-title": "Fold. Des."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB16",
      "doi-asserted-by": "crossref",
      "first-page": "475",
      "DOI": "10.1016/0092-8674(95)90436-0",
      "article-title": "The HIV-1 Rev activation domain is a nuclear export signal that accesses an export pathway used by specific cellular RNAs",
      "volume": "82",
      "author": "Fischer",
      "year": "1995",
      "journal-title": "Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB17",
      "doi-asserted-by": "crossref",
      "first-page": "25",
      "DOI": "10.1016/S0896-6273(03)00594-4",
      "article-title": "Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila",
      "volume": "40",
      "author": "Gunawardena",
      "year": "2003",
      "journal-title": "Neuron"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB18",
      "doi-asserted-by": "crossref",
      "first-page": "25",
      "DOI": "10.1093/hmg/8.1.25",
      "article-title": "In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease",
      "volume": "8",
      "author": "Hackam",
      "year": "1999",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB19",
      "doi-asserted-by": "crossref",
      "first-page": "710",
      "DOI": "10.1126/science.1079694",
      "article-title": "Spongiform degeneration in mahoganoid mutant mice",
      "volume": "299",
      "author": "He",
      "year": "2003",
      "journal-title": "Science"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB20",
      "doi-asserted-by": "crossref",
      "first-page": "425",
      "DOI": "10.1146/annurev.biochem.67.1.425",
      "article-title": "The ubiquitin system",
      "volume": "67",
      "author": "Hershko",
      "year": "1998",
      "journal-title": "Annu. Rev. Biochem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB21",
      "doi-asserted-by": "crossref",
      "first-page": "4307",
      "DOI": "10.1038/sj.emboj.7600426",
      "article-title": "Inefficient degradation of truncated polyglutamine proteins by the proteasome",
      "volume": "23",
      "author": "Holmberg",
      "year": "2004",
      "journal-title": "EMBO J."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB22",
      "doi-asserted-by": "crossref",
      "first-page": "34150",
      "DOI": "10.1074/jbc.M204955200",
      "article-title": "A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system",
      "volume": "277",
      "author": "Illing",
      "year": "2002",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB23",
      "doi-asserted-by": "crossref",
      "first-page": "1049",
      "DOI": "10.1093/hmg/10.10.1049",
      "article-title": "Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release",
      "volume": "10",
      "author": "Jana",
      "year": "2001",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB24",
      "doi-asserted-by": "crossref",
      "first-page": "12571",
      "DOI": "10.1073/pnas.220417997",
      "article-title": "Formation of high molecular weight complexes of mutant cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis",
      "volume": "97",
      "author": "Johnston",
      "year": "2000",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB25",
      "doi-asserted-by": "crossref",
      "first-page": "33",
      "DOI": "10.1038/311033a0",
      "article-title": "Sequence requirements for nuclear location of simian virus 40 large-T antigen",
      "volume": "311",
      "author": "Kalderon",
      "year": "1984",
      "journal-title": "Nature"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB26",
      "doi-asserted-by": "crossref",
      "first-page": "727",
      "DOI": "10.1016/S0092-8674(03)00939-5",
      "article-title": "The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress",
      "volume": "115",
      "author": "Kawaguchi",
      "year": "2003",
      "journal-title": "Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB27",
      "doi-asserted-by": "crossref",
      "first-page": "486",
      "DOI": "10.1126/science.1079469",
      "article-title": "Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis",
      "volume": "300",
      "author": "Kayed",
      "year": "2003",
      "journal-title": "Science"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB28",
      "doi-asserted-by": "crossref",
      "first-page": "11404",
      "DOI": "10.1073/pnas.96.20.11404",
      "article-title": "Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells",
      "volume": "96",
      "author": "Kazantsev",
      "year": "1999",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB29",
      "doi-asserted-by": "crossref",
      "first-page": "826",
      "DOI": "10.1038/ncb863",
      "article-title": "Polyglutamine protein aggregates are dynamic",
      "volume": "4",
      "author": "Kim",
      "year": "2002",
      "journal-title": "Nat. Cell Biol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB30",
      "doi-asserted-by": "crossref",
      "first-page": "41",
      "DOI": "10.1016/S0092-8674(00)81781-X",
      "article-title": "Ataxin-1 nuclear localization and aggregation",
      "volume": "95",
      "author": "Klement",
      "year": "1998",
      "journal-title": "Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB31",
      "doi-asserted-by": "crossref",
      "first-page": "9989",
      "DOI": "10.1073/pnas.96.18.9989",
      "article-title": "Amyloid diseases",
      "volume": "96",
      "author": "Koo",
      "year": "1999",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB32",
      "doi-asserted-by": "crossref",
      "first-page": "524",
      "DOI": "10.1016/S0962-8924(00)01852-3",
      "article-title": "Aggresomes, inclusion bodies and protein aggregation",
      "volume": "10",
      "author": "Kopito",
      "year": "2000",
      "journal-title": "Trends Cell Biol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB33",
      "doi-asserted-by": "crossref",
      "first-page": "291",
      "DOI": "10.1038/418291a",
      "article-title": "Neurodegenerative disease",
      "volume": "418",
      "author": "Lashuel",
      "year": "2002",
      "journal-title": "Nature"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB34",
      "doi-asserted-by": "crossref",
      "first-page": "477",
      "DOI": "10.1097/00019052-200108000-00008",
      "article-title": "Parkin and Parkinson's disease",
      "volume": "14",
      "author": "Mizuno",
      "year": "2001",
      "journal-title": "Curr. Opin. Neurol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB35",
      "doi-asserted-by": "crossref",
      "first-page": "2423",
      "DOI": "10.1126/science.1056784",
      "article-title": "Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity",
      "volume": "291",
      "author": "Nucifora",
      "year": "2001",
      "journal-title": "Science"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB36",
      "doi-asserted-by": "crossref",
      "first-page": "5355",
      "DOI": "10.1073/pnas.91.12.5355",
      "article-title": "Glutamine repeats as polar zippers",
      "volume": "91",
      "author": "Perutz",
      "year": "1994",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB37",
      "doi-asserted-by": "crossref",
      "first-page": "121",
      "DOI": "10.1006/mcne.1999.0773",
      "article-title": "Nuclear targeting of mutant Huntingtin increases toxicity",
      "volume": "14",
      "author": "Peters",
      "year": "1999",
      "journal-title": "Mol. Cell. Neurosci."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB38",
      "doi-asserted-by": "crossref",
      "first-page": "41032",
      "DOI": "10.1074/jbc.M205809200",
      "article-title": "Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization",
      "volume": "277",
      "author": "Poirier",
      "year": "2002",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB39",
      "doi-asserted-by": "crossref",
      "first-page": "6087",
      "DOI": "10.1093/emboj/16.20.6087",
      "article-title": "Dynamics of proteasome distribution in living cells",
      "volume": "16",
      "author": "Reits",
      "year": "1997",
      "journal-title": "EMBO J."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB40",
      "doi-asserted-by": "crossref",
      "first-page": "47",
      "DOI": "10.1038/12647",
      "article-title": "Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice",
      "volume": "23",
      "author": "Saigoh",
      "year": "1999",
      "journal-title": "Nat. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB41",
      "doi-asserted-by": "crossref",
      "first-page": "2907",
      "DOI": "10.1242/jcs.115.14.2907",
      "article-title": "The cellular fate of mutant rhodopsin",
      "volume": "115",
      "author": "Saliba",
      "year": "2002",
      "journal-title": "J. Cell Sci."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB42",
      "doi-asserted-by": "crossref",
      "first-page": "55",
      "DOI": "10.1016/S0092-8674(00)81782-1",
      "article-title": "Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions",
      "volume": "95",
      "author": "Saudou",
      "year": "1998",
      "journal-title": "Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB43",
      "doi-asserted-by": "crossref",
      "first-page": "95",
      "DOI": "10.1016/j.molcel.2004.06.029",
      "article-title": "Cellular toxicity of polyglutamine expansion proteins; mechanism of transcription factor deactivation",
      "volume": "15",
      "author": "Schaffar",
      "year": "2004",
      "journal-title": "Mol. Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB44",
      "doi-asserted-by": "crossref",
      "first-page": "4604",
      "DOI": "10.1073/pnas.96.8.4604",
      "article-title": "Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils",
      "volume": "96",
      "author": "Scherzinger",
      "year": "1999",
      "journal-title": "Proc. Natl. Acad. Sci. USA"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB45",
      "doi-asserted-by": "crossref",
      "first-page": "302",
      "DOI": "10.1002/ana.10101",
      "article-title": "Protein surveillance machinery in brains with spinocerebellar ataxia type 3",
      "volume": "51",
      "author": "Schmidt",
      "year": "2002",
      "journal-title": "Ann. Neurol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB46",
      "doi-asserted-by": "crossref",
      "first-page": "806",
      "DOI": "10.1038/ncb859",
      "article-title": "Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components",
      "volume": "4",
      "author": "Stenoien",
      "year": "2002",
      "journal-title": "Nat. Cell Biol."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB47",
      "doi-asserted-by": "crossref",
      "first-page": "81",
      "DOI": "10.1016/S1097-2765(04)00156-X",
      "article-title": "Caspase activation inhibits proteasome function during apoptosis",
      "volume": "14",
      "author": "Sun",
      "year": "2004",
      "journal-title": "Mol. Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB48",
      "doi-asserted-by": "crossref",
      "first-page": "26645",
      "DOI": "10.1016/S0021-9258(19)74360-9",
      "article-title": "Rhodopsin mutations responsible for autosomal dominant retinitis pigmentosa. Clustering of functional classes along the polypeptide chain",
      "volume": "268",
      "author": "Sung",
      "year": "1993",
      "journal-title": "J. Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB49",
      "doi-asserted-by": "crossref",
      "first-page": "1991",
      "DOI": "10.1126/science.1067122",
      "article-title": "Toxic proteins in neurodegenerative disease",
      "volume": "296",
      "author": "Taylor",
      "year": "2002",
      "journal-title": "Science"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB50",
      "doi-asserted-by": "crossref",
      "first-page": "749",
      "DOI": "10.1093/hmg/ddg074",
      "article-title": "Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein",
      "volume": "12",
      "author": "Taylor",
      "year": "2003",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB51",
      "doi-asserted-by": "crossref",
      "first-page": "355",
      "DOI": "10.1093/emboj/cdg044",
      "article-title": "From Alzheimer to Huntington",
      "volume": "22",
      "author": "Temussi",
      "year": "2003",
      "journal-title": "EMBO J."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB52",
      "doi-asserted-by": "crossref",
      "first-page": "194",
      "DOI": "10.1016/S0166-2236(99)01409-5",
      "article-title": "Aggregates in neurodegenerative disease",
      "volume": "22",
      "author": "Tran",
      "year": "1999",
      "journal-title": "Trends Neurosci."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB53",
      "doi-asserted-by": "crossref",
      "first-page": "95",
      "DOI": "10.1016/S1097-2765(04)00151-0",
      "article-title": "Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins",
      "volume": "14",
      "author": "Venkatraman",
      "year": "2004",
      "journal-title": "Mol. Cell"
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB54",
      "doi-asserted-by": "crossref",
      "first-page": "2689",
      "DOI": "10.1093/hmg/11.22.2689",
      "article-title": "Aggregate formation inhibits proteasomal degradation of polyglutamine proteins",
      "volume": "11",
      "author": "Verhoef",
      "year": "2002",
      "journal-title": "Hum. Mol. Genet."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB55",
      "doi-asserted-by": "crossref",
      "first-page": "937",
      "DOI": "10.1515/BC.2000.114",
      "article-title": "Protein aggregation and pathogenesis of Huntington's disease",
      "volume": "381",
      "author": "Wanker",
      "year": "2000",
      "journal-title": "Biol. Chem."
    },
    {
      "key": "10.1016/j.molcel.2004.12.021_BIB56",
      "doi-asserted-by": "crossref",
      "first-page": "375",
      "DOI": "10.1016/S0076-6879(99)09026-6",
      "article-title": "Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates",
      "volume": "309",
      "author": "Wanker",
      "year": "1999",
      "journal-title": "Methods Enzymol."
    }
  ],
  "container-title": "Molecular Cell",
  "original-title": [],
  "language": "en",
  "link": [
    {
      "URL": "https://api.elsevier.com/content/article/PII:S1097276505010087?httpAccept=text/xml",
      "content-type": "text/xml",
      "content-version": "vor",
      "intended-application": "text-mining"
    },
    {
      "URL": "https://api.elsevier.com/content/article/PII:S1097276505010087?httpAccept=text/plain",
      "content-type": "text/plain",
      "content-version": "vor",
      "intended-application": "text-mining"
    }
  ],
  "deposited": {
    "date-parts": [
      [
        2021,
        7,
        4
      ]
    ],
    "date-time": "2021-07-04T16:56:17Z",
    "timestamp": 1625417777000
  },
  "score": 1,
  "resource": {
    "primary": {
      "URL": "https://linkinghub.elsevier.com/retrieve/pii/S1097276505010087"
    }
  },
  "subtitle": [],
  "short-title": [],
  "issued": {
    "date-parts": [
      [
        2005,
        2
      ]
    ]
  },
  "references-count": 56,
  "journal-issue": {
    "issue": "3",
    "published-print": {
      "date-parts": [
        [
          2005,
          2
        ]
      ]
    }
  },
  "alternative-id": [
    "S1097276505010087"
  ],
  "URL": "http://dx.doi.org/10.1016/j.molcel.2004.12.021",
  "relation": {},
  "ISSN": [
    "1097-2765"
  ],
  "subject": [],
  "container-title-short": "Molecular Cell",
  "published": {
    "date-parts": [
      [
        2005,
        2
      ]
    ]
  }
}