Article
Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation.
The European journal of neuroscience - 1 Dec 2004
Ribchester Richard R, Thomson Derek, Wood Nigel I, Hinks Tim, Gillingwater Thomas H, Wishart Thomas M, Court Felipe A, Morton A Jennifer
Abstract excerpt
Huntington's disease (HD) is a neurodegenerative disorder with complex symptoms dominated by progressive motor dysfunction. Skeletal muscle atrophy is common in HD patients. Because the HD mutation is expressed in skeletal muscle as well as brain, we wondered whether the muscle changes arise from primary pathology. We used R6/2 transgenic mice for our studies. Unlike denervation atrophy, skeletal muscle atrophy...
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