FA2H and leukodystrophy: FA2H is responsible for adding a hydroxyl group to the C-2 position of free FAs to yield 2-hydroxy FAs, the products of which are further converted to a subset of sphingolipids and glycosphingolipids.11,12 FA2H and its catalytic products play vital roles in the epidermis and nervous system under physiological conditions, as exemplified by the observation that mutations in FA2H are implicated in spastic paraparesis and leukodystrophy.13 In recent years, it is becoming increasingly evident that FA2H also functions in several cancer types.