Proteases Acting on Mutant Huntingtin Generate Cleaved Products that Differentially Build Up Cytoplasmic and Nuclear Inclusions

Molecular Cell - Tập 10 Số 2 - Trang 259-269 - 2002
Astrid Lunkes1, Katrin S. Lindenberg2, Léa Ben-Haı̈em1, C. R. Weber1, Didier Devys1, G. Bernhard Landwehrmeyer2, Jean‐Louis Mandel1, Yvon Trottier1
1Institut de Génétique et de Biologie Moléculaire et Cellulaire
2University of Ulm

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Barrett, 2001, The MEROPS database as a protease information system, J. Struct. Biol., 134, 95, 10.1006/jsbi.2000.4332

Bence, 2001, Impairment of the ubiquitin-proteasome system by protein aggregation, Science, 292, 1552, 10.1126/science.292.5521.1552

Craig, 2002, A masked NES in INI1/hSNF5 mediates hCRM1-dependent nuclear export, EMBO J., 21, 31, 10.1093/emboj/21.1.31

Davies, 1997, Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation, Cell, 90, 537, 10.1016/S0092-8674(00)80513-9

Di Figlia, 1997, Aggregation of Huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain, Science, 277, 1990, 10.1126/science.277.5334.1990

Dorsman, 1999, Analysis of the subcellular localization of huntingtin with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin, Philos. Trans. R. Soc. Lond. B Biol. Sci., 354, 1061, 10.1098/rstb.1999.0459

Duncan, 1997, Cleavage site for sterol-regulated protease localized to a leu-Ser bond in the lumenal loop of sterol regulatory element-binding protein-2, J. Biol. Chem., 272, 12778, 10.1074/jbc.272.19.12778

Duncan, 1998, Second-site cleavage in sterol regulatory element-binding protein occurs at transmembrane junction as determined by cysteine panning, J. Biol. Chem., 273, 17801, 10.1074/jbc.273.28.17801

Dyer, 2001, Mutant protein in Huntington disease is resistant to proteolysis in affected brain, Nat. Genet., 29, 270, 10.1038/ng745

Gutekunst, 1999, Nuclear and neuropil aggregates in Huntington's disease, J. Neurosci., 19, 2522, 10.1523/JNEUROSCI.19-07-02522.1999

Hackam, 1998, The influence of huntingtin protein size on nuclear localization and cellular toxicity, J. Cell Biol., 141, 1097, 10.1083/jcb.141.5.1097

Hackam, 1999, In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease, Hum. Mol. Genet., 8, 25, 10.1093/hmg/8.1.25

Hazeki, 2000, Formic acid dissolves aggregates of an N-terminal huntingtin fragment containing an expanded polyglutamine tract, Biochem. Biophys. Res. Commun., 277, 386, 10.1006/bbrc.2000.3682

Hodgson, 1999, A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration, Neuron, 23, 181, 10.1016/S0896-6273(00)80764-3

Jana, 2001, Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release, Hum. Mol. Genet., 10, 1049, 10.1093/hmg/10.10.1049

Kegel, 2000, Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy, J. Neurosci., 20, 7268, 10.1523/JNEUROSCI.20-19-07268.2000

Kim, 2001, Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis, Proc. Natl. Acad. Sci. USA, 98, 12784, 10.1073/pnas.221451398

Klement, 1998, Ataxin-1 nuclear localization and aggregation, Cell, 95, 41, 10.1016/S0092-8674(00)81781-X

Lichtenthaler, 1999, Mechanism of the cleavage specificity of Alzheimer's disease gamma-secretase identified by phenylalanine-scanning mutagenesis of the transmembrane domain of the amyloid precursor protein, Proc. Natl. Acad. Sci. USA, 96, 3053, 10.1073/pnas.96.6.3053

Lunkes, 1998, A cellular model that recapitulates major pathogenic steps of Huntington's disease, Hum. Mol. Genet., 7, 1355, 10.1093/hmg/7.9.1355

Lunkes, 2000, Cellular models of Huntington's disease, Neuroscience News, 3, 30

Lunkes, 1999, Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease, Philos. Trans. R. Soc. Lond. B Biol. Sci., 354, 1013, 10.1098/rstb.1999.0453

Mende-Mueller, 2001, Tissue-specific proteolysis of Huntingtin (htt) in human brain, J. Neurosci., 21, 1830, 10.1523/JNEUROSCI.21-06-01830.2001

Nakamura, 2001, SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein, Hum. Mol. Genet., 10, 1441, 10.1093/hmg/10.14.1441

Perez, 1998, Recruitment and the role of nuclear localisation in polyglutamine-mediated aggregation, J. Cell Biol., 14, 1457, 10.1083/jcb.143.6.1457

Perutz, 1994, Glutamine repeats as polar zippers, Proc. Natl. Acad. Sci. USA, 91, 5355, 10.1073/pnas.91.12.5355

Scherzinger, 1997, Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo, Cell, 90, 549, 10.1016/S0092-8674(00)80514-0

Sisodia, 1992, Beta-amyloid precursor protein cleavage by a membrane-bound protease, Proc. Natl. Acad. Sci. USA, 89, 6075, 10.1073/pnas.89.13.6075

Steffan, 2000, The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription, Proc. Natl. Acad. Sci. USA, 97, 6763, 10.1073/pnas.100110097

Suhr, 2001, Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression, J. Cell Biol., 153, 283, 10.1083/jcb.153.2.283

Trottier, 1995, Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form, Nat. Genet., 10, 104, 10.1038/ng0595-104

Trottier, 1995, Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias, Nature, 378, 403, 10.1038/378403a0

Vonsattel, 1985, Neuropathological classification of Huntington's disease, J. Neuropathol. Exp. Neurol., 44, 559, 10.1097/00005072-198511000-00003

Wellington, 2000, Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells, J. Biol. Chem., 275, 19831, 10.1074/jbc.M001475200

Wheeler, 2000, Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice, Hum. Mol. Genet., 9, 503, 10.1093/hmg/9.4.503

Yamamoto, 2000, Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease, Cell, 101, 57, 10.1016/S0092-8674(00)80623-6

Zoghbi, 2000, Glutamine repeats and neurodegeneration, Annu. Rev. Neurosci., 23, 217, 10.1146/annurev.neuro.23.1.217