Myogenic contributions to SBMA were first recognized in two different mouse models (Yu et al., 2006; Monks et al., 2007), where myopathic changes were discovered in a knock-in model well before neuropathy and transgenically overexpressed WT-AR specifically in muscle cells triggered SBMA-like symptoms only in myogenic TG males, including a loss of ventral root axons. The gene discussed is AR; the disease is Kennedy disease.