Anti-HD-polySer-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. PolySer is a sense repeat-associated non-ATG (RAN) translation protein that accumulates in various brain regions with toxic effects leading to microglial activation and neuronal loss. HD-RAN polySer toxicity is shown to be comparable to that of polyGln. Aggregation of polySer RAN proteins is shown in cells expressing longer repeats greater than 52, which are typically associated with earlier disease onset and in more severe juvenile HD. 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.