Enzyme activity of both glucosylceramide synthase (GCS, encoded by the UGCG gene) and of non-lysosomal glucosylceramidases (GBA2) in spinal cord and muscle are increased from the beginning of denervation in multiple animal models of ALS [63,64,65,66,67,68,69]. The gene discussed is GBA2; the disease is amyotrophic lateral sclerosis.