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Huntingtons disease (HD) is an autosomal dominant neurodegenerative disorder caused by cytotoxicity of the mutant huntingtin (mHtt) protein.While mHtt is ubiquitously expressed, HD is characterized by selective degeneration of the corpus striatum, which is partially caused by a slower clearance rate of mHtt in the striatal neurons, suggesting existence of striatal-enriched mHtt stabilizers.