Recently, it has been discovered that the mutations in the SPTLC1 subunit of serine palmitoyltransferase (SPT) catalyzes the first step of de novo synthesis of sphingolipids and leads to the childhood-onset ALS (Johnson et al., 2021; Mohassel et al., 2021). The gene discussed is SPTLC1; the disease is amyotrophic lateral sclerosis.