Using a mouse model of SBMA that carries a pathogenic 100 glutamine repeat in the androgen receptor (AR100) and which develops a late onset neuromuscular phenotype, accompanied by motor neuron degeneration (Sopher et al., 2004; Malik et al., 2011, 2013) resembling the human disease, we examined the role of endoplasmic reticulum stress in motor neuron death in SBMA. This evidence concerns the gene AR and Kennedy disease.