SOD1 and proximal spinal muscular atrophy: Similarly, abnormal glycosphingolipid metabolism, a feature of SMA, HSP, and LSDs, was also demonstrated in the ALS1 transgenic mice SOD1G93A and cells from patients, and the dysregulation of glucosylceramide content (a sign of LSDs) was also shown in the ALS1 transgenic mice SODG86R [6].