Article
Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures.
Nature - 30 Oct 1997
Skinner P J, Koshy B T, Cummings C J, Klement I A, Helin K, Servadio A, Zoghbi H Y, Orr H T
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is one of several neurodegenerative disorders caused by an expansion of a polyglutamine tract. It is characterized by ataxia, progressive motor deterioration, and loss of cerebellar Purkinje cells. To understand the pathogenesis of SCA1, we examined the subcellular localization of wild-type human ataxin-1 (the protein encoded by the SCA1 gene) and mutant ataxin-1 in the...
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