Anti-HD-polyCys-Ct serum, from rabbit

Huntington disease (HD) is a progressive neurodegenerative disorder is caused by a CAG,CTG expansion in the HTT gene that results in the production of a mutant huntingtin protein (HTT) with polymeric expansions that accumulate in human brain with HD. PolyCys is an antisense repeat-associated non-ATG (RAN) translation protein that accumulate in various brain regions with toxic effects leading to microglial activation and neuronal loss. HD-RAN polyCys toxicity is shown to be comparable to that of polyGln. HD-RAN proteins are abundant in regions of the brain that are most affected by HD and these regions display pathologic features of HD, such as caspase-3 activity and microglial activation. HD-Ran protein accumulation and aggregation in HD brains are shown to be length dependent.