Document Detail


USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product.
MedLine Citation:
PMID:  12093161     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Sunghoi Hong; Sung-Jo Kim; Sojeong Ka; Inho Choi; Seongman Kang
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Molecular and cellular neurosciences     Volume:  20     ISSN:  1044-7431     ISO Abbreviation:  Mol. Cell. Neurosci.     Publication Date:  2002 Jun 
Date Detail:
Created Date:  2002-07-02     Completed Date:  2002-08-29     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9100095     Medline TA:  Mol Cell Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  298-306     Citation Subset:  IM    
Copyright Information:
(c) 2002 Elsevier Science (USA).
Affiliation:
Graduate School of Biotechnology, Korea University, 1,5-ka Anam-dong, Sungbuk-ku, Seoul 136-701, Korea.
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MeSH Terms
Descriptor/Qualifier:
Animals
COS Cells
Cerebellum / abnormalities*,  metabolism,  pathology
Endopeptidases / genetics,  isolation & purification,  metabolism*
Immunohistochemistry
Inclusion Bodies / metabolism,  pathology
Mice
Mice, Transgenic
Mutation / genetics
Nerve Tissue Proteins / genetics,  metabolism*
Neurons / metabolism*,  pathology
Nuclear Proteins / genetics,  metabolism*
Peptides / metabolism
Protein Binding / physiology
Purkinje Cells / metabolism,  pathology
Spinocerebellar Ataxias / enzymology*,  genetics,  physiopathology
Trinucleotide Repeat Expansion / genetics
Two-Hybrid System Techniques
Ubiquitin Thiolesterase
Chemical
Reg. No./Substance:
0/Nerve Tissue Proteins; 0/Nuclear Proteins; 0/Peptides; 0/ataxin-1; 26700-71-0/polyglutamine; EC 3.1.2.15/USP7 protein, human; EC 3.1.2.15/Ubiquitin Thiolesterase; EC 3.4.-/Endopeptidases; EC 3.4.99.-/ubiquitin-specific protease

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