ExplorEnz: EC 1.14.18.6

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1.14.18.6

Accepted name:

4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase

Reaction:

a phytoceramide + 2 ferrocytochrome b₅ + O₂ + 2 H⁺ = a (2′R)-2′-hydroxyphytoceramide + 2 ferricytochrome b₅ + H₂O

Glossary:

a phytoceramide = a (4R)-4-hydroxysphinganine ceramide = an N-acyl-4-hydroxysphinganine

Other name(s):

FA2H (gene name); SCS7 (gene name)

Systematic name:

(4R)-4-hydroxysphinganine ceramide,ferrocytochrome-b₅:oxygen oxidoreductase (fatty acyl 2-hydroxylating)

Comments:

The enzyme, characterized from yeast and mammals, catalyses the hydroxylation of carbon 2 of long- or very-long-chain fatty acids attached to (4R)-4-hydroxysphinganine during de novo ceramide synthesis. The enzymes from yeast and from mammals contain an N-terminal cytochrome b₅ domain that acts as the direct electron donor to the desaturase active site. The newly introduced 2-hydroxyl group has R-configuration. cf. EC 1.14.18.7, dihydroceramide fatty acyl 2-hydroxylase.

Links to other databases:

BRENDA, EXPASY, KEGG, MetaCyc, PDB

References:

1.	Mitchell, A.G. and Martin, C.E. Fah1p, a Saccharomyces cerevisiae cytochrome b₅ fusion protein, and its Arabidopsis thaliana homolog that lacks the cytochrome b₅ domain both function in the α-hydroxylation of sphingolipid-associated very long chain fatty acids. J. Biol. Chem. 272 (1997) 28281–28288. [DOI] [PMID: 9353282]
2.	Dunn, T.M., Haak, D., Monaghan, E. and Beeler, T.J. Synthesis of monohydroxylated inositolphosphorylceramide (IPC-C) in Saccharomyces cerevisiae requires Scs7p, a protein with both a cytochrome b₅-like domain and a hydroxylase/desaturase domain. Yeast 14 (1998) 311–321. [DOI] [PMID: 9559540]
3.	Alderson, N.L., Rembiesa, B.M., Walla, M.D., Bielawska, A., Bielawski, J. and Hama, H. The human FA2H gene encodes a fatty acid 2-hydroxylase. J. Biol. Chem. 279 (2004) 48562–48568. [DOI] [PMID: 15337768]
4.	Eckhardt, M., Yaghootfam, A., Fewou, S.N., Zoller, I. and Gieselmann, V. A mammalian fatty acid hydroxylase responsible for the formation of α-hydroxylated galactosylceramide in myelin. Biochem. J. 388 (2005) 245–254. [DOI] [PMID: 15658937]
5.	Guo, L., Zhang, X., Zhou, D., Okunade, A.L. and Su, X. Stereospecificity of fatty acid 2-hydroxylase and differential functions of 2-hydroxy fatty acid enantiomers. J. Lipid Res. 53 (2012) 1327–1335. [DOI] [PMID: 22517924]

[EC 1.14.18.6 created 2015]