Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 (original) (raw)
Wisniewski, T., Ghiso, J. & Frangione, B. Biology of A beta amyloid in Alzheimer's disease. Neurobiol. Dis.4, 313–328 (1997) ArticleCASPubMed Google Scholar
Polymeropoulos, M.H. et al. Mutation in the α-Synuclein Gene Identified in Families with Parkinson's Disease. Science276, 2045–2047 (1997) ArticleCASPubMed Google Scholar
Davies, S.W. et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell90, 537–548 (1997) ArticleCASPubMed Google Scholar
DiFiglia, M. et al. Aggregation of Huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science277, 1990–1993 (1997) ArticleCASPubMed Google Scholar
Igarashi, S. et al. Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nature Genet.18, 111–117 (1998) ArticleCASPubMed Google Scholar
Skinner, P.J. et al. Ataxin-1 with extra glutamines induces alterations in nuclear matrix-associated structures. Nature389, 971–974 (1997) ArticleCASPubMed Google Scholar
Paulson, H.L. et al. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia Type 3. Neuron19, 333–334 (1997) ArticleCASPubMed Google Scholar
Perutz, M.F., Johnson, T., Suzuki, M. & Finch, J.T. Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases . Proc. Natl. Acad. Sci. USA91, 5355–5358 (1994) ArticleCASPubMedPubMed Central Google Scholar
Stott, K., Blackburn, J.M., Butler, P.J.G. & Perutz, M. Incorporation of glutamine repeats makes protein oligomerize: Implications for neurodegenerative diseases. Proc. Natl. Acad. Sci. USA92, 6509–6513 (1995) ArticleCASPubMedPubMed Central Google Scholar
Hershko, A. & Ciechanover, A. The ubiquitin system for protein degradation. Annu. Rev. Biochem.61, 761 –807 (1992) ArticleCASPubMed Google Scholar
Hochstrasser, M. Ubiquitin-dependent protein degradation. Annu. Rev. Genet.30, 405–439 (1996) ArticleCASPubMed Google Scholar
Adams, G.M. et al. Structural and Functional Effects of PA700 and Modular Protein on Proteasomes . J. Mol. Biol.273, 646–657 (1997) ArticleCASPubMed Google Scholar
Coux, O., Tanaka, K. & Goldberg, A.L. Structure and functions of the 20S and 26S proteasomes . Annu. Rev. Biochem.65, 801–847 (1996) ArticleCASPubMed Google Scholar
Bush, K.T., Goldberg, A.L. & Nigam, S.K. Proteasome Inhibition Leads to a Heat-shock Response, Induction of Endoplasmic Reticulum Chaperones, and Thermotolerance. J. Biol. Chem.272, 9086–9092 (1997) ArticleCASPubMed Google Scholar
Zhou, M., Wu, X. & Ginsberg, H.N. Evidence that a rapidly turning over protein, normally degraded by proteasomes, regulates hsp72 gene transcription in HepG2 cells. J. Biol. Chem.271, 247–269 ( 1996) Google Scholar
Lee, D.H. & Goldberg, A.L. Proteasome Inhibitors Cause Induction of Heat Shock Proteins and Trehalose, Which Together Confer Thermotolerance in Saccharomyces cerevisiae. Mol. Cell. Biol.18, 30–38 (1998) ArticleCASPubMedPubMed Central Google Scholar
Lu, Z. & Cyr, D.M. The Conserved Carboxyl Terminus and Zinc Finger-Like Domain of the Co-chaperone Ydj1 Assist Hsp70 in Protein Folding . J. Biol. Chem.273, 5970–5978 (1998) ArticleCASPubMed Google Scholar
Hendricks, J.P. & Hartl, F. -U.Molecular chaperone functions of heat shock proteins. Annu. Rev. Biochem.62, 349–384 (1993) Article Google Scholar
Sherman, M.Y. & Goldberg, A.L. Involvement of chaperonin dnaK in the rapid degradation of a mutant protein in Escherichia coli. EMBO J.11, 71–77 ( 1992) CAS Google Scholar
Straus, D.B., Walter, W.A. & Gross, C.A. Escherichia coli heat shock gene mutants are defective in proteolysis . Genes Dev.2, 1851–1858 (1988) ArticleCASPubMed Google Scholar
Lee, D.H., Sherman, M.Y. & Goldberg, A.L. Involvement of the Molecular Chaperone Ydj1 in the Ubiquitin-Dependent Degradation of Short-Lived and Abnormal Proteins in Saccharomyces cerevisiae. Mol. Cell. Biol.16, 4773–4781 (1996) ArticleCASPubMedPubMed Central Google Scholar
Burright, E.N. et al. SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell82, 937–948 (1995) ArticleCASPubMed Google Scholar
Attaix, D. et al. Expression of subunits of the 19S complex and of the PA28 activator in rat skeletal muscle. Mol. Biol. Rep.24, 95–98 (1997) ArticleCASPubMed Google Scholar
Chellaiah, A., Davis, A. & Mohanakumar, T. Cloning of a unique human homologue of the Escherichia coli DANJ heat shock protein. Biochim. Biophys. Acta1174, 111–113 (1993) ArticleCASPubMed Google Scholar
Oh, S., Iwahori, A. & Kato, S. Human cDNA encoding DnaJ protein homologue. Biochim. Biophys. Acta1174, 114–116 ( 1993) ArticleCASPubMed Google Scholar
Cyr, D.M., Lu, X. & Douglas, M.G. Regulation of Hsp70 function by a eukaryotic DnaJ homolog. J. Biol. Chem.267, 20927–20931 ( 1992) CASPubMed Google Scholar
Tang, Y., Ramakrishnan, C., Thomas, J. & DeFranco, D.B. A Role for HDJ-2/HSDJ in Correcting Subnuclear Trafficking, Transactivation, and Transrepression Defects of a Glucocorticoid Receptor Zinc Finger Mutant. Mol. Biol. Cell8, 795–809 ( 1997) ArticleCASPubMedPubMed Central Google Scholar
Schirmer, E.C. & Lindquist, S. Interactions of the chaperone Hsp104 with yeast Sup35 and mammalian PrP. Proc. Natl. Acad. Sci. USA94, 13932–13937 ( 1997) ArticleCASPubMedPubMed Central Google Scholar
DebBurman, S.K., Raymond, G.J., Caughey, B. & Lindquist, S. Chaperone-supervised conversion of prion protein to its protease-resistant form. Proc. Natl. Acad. Sci. USA94, 13938 –13943 (1997) ArticleCASPubMedPubMed Central Google Scholar
Chernoff, Y.O., Lindquist, S.L., Ono, B., Inge-Vechtomov, S.G. & Liebman, S.W. Role of the Chaperone Protein Hsp104 in Propagation of the Yeast Prion-Like Factor [_psi+_]. Science268, 880–883 (1995) ArticleCASPubMed Google Scholar
Cyr, D.M., Langer, T. & Douglas, M.G. DnaJ-like proteins: molecular chaperones and specific regulators of Hsp70 . Trends Biochem. Sci.19, 176–181 (1994) ArticleCASPubMed Google Scholar
Dienel, G., Kiessling, M., Soubrie, P., Bockaert, J. & Pin, J. Synthesis of heat shock proteins in rat brain cortex after transient ischemia. J. Cereb. Blood Flow Metab.6, 505–510 (1986) ArticleCASPubMed Google Scholar
Brown, I.R. Induction of heat shock (stress) genes in the mammalian brain by hyperthermia and other traumatic events: a current perspective. J. Neurosci. Res.27, 247–255 (1990) ArticleCASPubMed Google Scholar
Cyr, D.M. Cooperation of the molecular chaperone Ydj1 with specific Hsp70 homologs to suppress protein aggregation. FEBS Lett.359, 129–132 (1995) ArticleCASPubMed Google Scholar
Schumacher, R.J. et al. Cooperative action of Hsp70, Hsp90, and DnaJ proteins in protein renaturation . Biochemistry35, 14889–14898 (1996) ArticleCASPubMed Google Scholar
Matilla, T. et al. The cerebellar leucine rich acidic nuclear protein interacts with ataxin-1 . Nature389, 974–978 (1997) ArticleCASPubMed Google Scholar
Shibatani, T. & Ward, W.F. Sodium dodecyl sulfate (SDS) activation of the 20S proteasome in rat liver. Arch. Biochem. Biophys.321, 160–166 (1995) ArticleCASPubMed Google Scholar
Servadio, A. et al. Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nature Genet.10, 94–98 (1995) ArticleCASPubMed Google Scholar