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]. Here, SOD1 is linked to hereditary spastic paraplegia.