Article
Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.
Neuron - 29 Aug 2002
Katsuno Masahisa, Adachi Hiroaki, Kume Akito, Li Mei, Nakagomi Yuji, Niwa Hisayoshi, Sang Chen, Kobayashi Yasushi, Doyu Manabu, Sobue Gen
Abstract excerpt
Spinal and bulbar muscular atrophy (SBMA) is a polyglutamine disease caused by the expansion of a CAG repeat in the androgen receptor (AR) gene. We generated a transgenic mouse model carrying a full-length AR containing 97 CAGs. Three of the five lines showed progressive muscular atrophy and weakness as well as diffuse nuclear staining and nuclear inclusions consisting of the mutant AR. These phenotypes were...
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