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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
2
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pubmed:dateCreated |
2002-7-2
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pubmed:abstractText |
Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 has been known to associate with elongated polyglutamine tract in ataxin-1, the SCA1 gene product. Using the yeast two-hybrid system, we have found that USP7, a ubiquitin-specific protease, binds to ataxin-1. Further experiments with deletion mutants indicated that the C-terminal region of ataxin-1 was essential for the interaction. Liquid beta-galactosidase assay and coimmunoprecipitation experiments revealed that the strength of the interaction between USP7 and ataxin-1 is influenced by the length of the polyglutamine tract in the ataxin-1; weaker interaction was observed in mutant ataxin-1 with longer polyglutamine tract and USP7 was not recruited to the mutant ataxin-1 aggregates in the Purkinje cells of SCA1 transgenic mice. Our results suggest that altered function of the ubiquitin system can be involved in the pathogenesis of spinocerebellar ataxia type 1.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Jun
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pubmed:issn |
1044-7431
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pubmed:author |
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pubmed:copyrightInfo |
(c) 2002 Elsevier Science (USA).
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pubmed:issnType |
Print
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pubmed:volume |
20
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
298-306
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pubmed:dateRevised |
2007-11-15
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pubmed:meshHeading |
pubmed-meshheading:12093161-Animals,
pubmed-meshheading:12093161-COS Cells,
pubmed-meshheading:12093161-Cerebellum,
pubmed-meshheading:12093161-Endopeptidases,
pubmed-meshheading:12093161-Immunohistochemistry,
pubmed-meshheading:12093161-Inclusion Bodies,
pubmed-meshheading:12093161-Mice,
pubmed-meshheading:12093161-Mice, Transgenic,
pubmed-meshheading:12093161-Mutation,
pubmed-meshheading:12093161-Nerve Tissue Proteins,
pubmed-meshheading:12093161-Neurons,
pubmed-meshheading:12093161-Nuclear Proteins,
pubmed-meshheading:12093161-Peptides,
pubmed-meshheading:12093161-Protein Binding,
pubmed-meshheading:12093161-Purkinje Cells,
pubmed-meshheading:12093161-Spinocerebellar Ataxias,
pubmed-meshheading:12093161-Trinucleotide Repeat Expansion,
pubmed-meshheading:12093161-Two-Hybrid System Techniques,
pubmed-meshheading:12093161-Ubiquitin Thiolesterase
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pubmed:year |
2002
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pubmed:articleTitle |
USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product.
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pubmed:affiliation |
Graduate School of Biotechnology, Korea University, 1,5-ka Anam-dong, Sungbuk-ku, Seoul 136-701, Korea.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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