Transcriptional Profile of Muscle following Acute Induction of Symptoms in a Mouse Model of Kennedy's Disease/Spinobulbar Muscular Atrophy

BackgroundKennedy’s disease/Spinobulbar muscular atrophy (KD/SBMA) is a degenerativeneuromuscular disease affecting males. This disease is caused by polyglutamineexpansion mutations of the androgen receptor (AR) gene. AlthoughKD/SBMA has been traditionally considered a motor neuron disease, emergingevidence points to a central etiological role of muscle. We previously reported amicroarray study of genes differentially expressed in muscle of three geneticallyunique mouse models of KD/SBMA but were unable to detect those which areandrogen-dependent or are associated with onset of symptoms.Conclusions/SignificanceBy comparing the current results with those from the three previously reportedmodels we were able to identify KD/SBMA candidate genes that are androgendependent, and occur early in the disease process, properties which are promisingfor targeted therapeutics.