Article
Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 1 Oct 1997
Clark H B, Burright E N, Yunis W S, Larson S, Wilcox C, Hartman B, Matilla A, Zoghbi H Y, Orr H T
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurological disorder caused by the expansion of a CAG repeat encoding a polyglutamine tract. Work presented here describes the behavioral and neuropathological course seen in mutant SCA1 transgenic mice. Behavioral tests indicate that at 5 weeks of age mutant mice have an impaired performance on the rotating rod in the absence of deficits in balance...
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