InterPro domain: IPR014430

This entry represents a family of fatty acid hydroxylases, including Scs7 from budding yeasts and FA2H from animals and plants. They contain an N-terminal Cytochrome b5-like heme/steroid binding domain.

Scs7 is a sphingolipid alpha-hydroxylase involved in the alpha-hydroxylation of sphingolipid-associated very long chain fatty acids [ 1 ].

Human FA2H is required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids [ 2 ]. It is is responsible for the formation of alpha-hydroxylated ceramide in oligodendrocytes of the mammalian brain [ 3 ].

1. Fah1p, a Saccharomyces cerevisiae cytochrome b5 fusion protein, and its Arabidopsis thaliana homolog that lacks the cytochrome b5 domain both function in the alpha-hydroxylation of sphingolipid-associated very long chain fatty acids. J. Biol. Chem. 272, 28281-8
2. The human FA2H gene encodes a fatty acid 2-hydroxylase. J. Biol. Chem. 279, 48562-8
3. A mammalian fatty acid hydroxylase responsible for the formation of alpha-hydroxylated galactosylceramide in myelin. Biochem. J. 388, 245-54