AGXT and amyotrophic lateral sclerosis: Dihydroceramide accumulation may also contribute to other neurodegenerative diseases, as gain of function mutations in components of the SPT complex cause juvenile amyotrophic lateral sclerosis due to elevated sphingolipid biosynthesis, with dihydroceramides showing the greatest relative increase of all sphingolipids (Lone et al., 2022; Lone et al., 2023; Syeda et al., 2024; Dohrn et al., 2024; Srivastava et al., 2023).