Article
Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model.
The Journal of clinical investigation - 1 Oct 2006
Yu Zhigang, Dadgar Nahid, Albertelli Megan, Gruis Kirsten, Jordan Cynthia, Robins Diane M, Lieberman Andrew P
Abstract excerpt
Kennedy disease, a degenerative disorder characterized by androgen-dependent neuromuscular weakness, is caused by a CAG/glutamine tract expansion in the androgen receptor (Ar) gene. We developed a mouse model of Kennedy disease, using gene targeting to convert mouse androgen receptor (AR) to human sequence while introducing 113 glutamines. AR113Q mice developed hormone and glutamine length-dependent neuromuscular...
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