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Publication : The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1).

First Author  Orr HT Year  2000
Journal  Neurobiol Dis Volume  7
Issue  3 Pages  129-34
PubMed ID  10860780 Mgi Jnum  J:64965
Mgi Id  MGI:1891527 Doi  10.1006/nbdi.2000.0305
Citation  Orr HT (2000) The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1). Neurobiol Dis 7(3):129-34
abstractText  Polyglutamine neurodegenerative disorders are characterized by the expansion of a glutamine tract within the mutant disease-causing protein. Expression of the mutant protein induces a progressive loss of neuronal function and the subsequent neurodegeneration of a set of neurons characteristic to each disease. Spinocerebellar ataxia type 1 (SCA1) is one polyglutamine disease where various experimental model systems, in particular transgenic mice, have been utilized to dissect the molecular and cellular events important for disease. This review summarizes these findings and places them in a context of potential future research directions. Copyright 2000 Academic Press.
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