Article
DRPLA: recent advances in research using transgenic mouse models.
Methods in molecular biology (Clifton, N.J.) - 1 Jan 2013
Suzuki Kazushi, Sato Toshiya, Yamada Mitsunori, Takahashi Hitoshi, Tsuji Shoji
Abstract excerpt
Dentatorubral-pallidoluysian atrophy (DRPLA) is one form of trinucleotide repeat disease, which has a high prevalence rate in the Japanese population. Our group established DRPLA transgenic mice harboring a single copy of a full-length human mutant DRPLA gene with 76 CAG repeats (Q76 mice). Q76 mice showed no obvious neurological phenotype but showed somatic and intergenerational instabilities of CAG repeats...
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