CASP6 and Huntington disease: Several HD mouse models have been used to demonstrate that alterations in protein sequence markedly suppress the phenotype of HD, including the YAC128 transgenic mice “short stop” (a mutation prevents expression of full length protein), the YAC128 line in which the caspase -6 cleavage site has been eliminated, and the BAC transgenic mice expressing full-length mutant huntingtin with serines 13 and 16 mutated to aspartate37, 38, 39.