A similar mechanism is causative for childhood ALS, in that variants of two subunits of SPT, SPTLC1 [163] and SPTLC2 [164] (the gate-keepers of GSL synthesis) favour changes in substrate from serine to alanine or glycine, increasing the formation of deoxysphingolipids (including 1-deoxyceramide), which are more resistant to degradation [163]. This evidence concerns the gene SPTLC1 and amyotrophic lateral sclerosis.