Reactome | FA2H hydroxylates 1,2-saturated fatty acids

FA2H hydroxylates 1,2-saturated fatty acids

Stable Identifier

R-HSA-5693761

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum membrane, cytosol

ReviewStatus

5/5

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FA2H hydroxylates 1,2-saturated fatty acids

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Fatty acid 2-hydroxylase (FA2H), an ER membrane-associated enzyme, catalyzes the cytochrome b5-dependent hydroxylation of free fatty acids on the saturated C2 position. 2-Hydroxylation of free fatty acids occurs before de novo ceramide synthesis. In mammals, 2-hydroxysphingolipids are abundant in the brain as components of the myelin lipids galactosylceramides and sulfatides (Alderson et al., 2004; Uchida et al., 2007; Guo et al., 2012; reviewed by Hama, 2009). Deficiency in FA2H causes a form of spastic paraplegia (SPG35, MIM:612319). A low expression level of FA2H correlates with a poor prognosis in many cancers (reviewed by Eckhardt, 2023).

Literature References

PubMed ID	Title	Journal	Year
20026285	Fatty acid 2-Hydroxylation in mammalian sphingolipid biology Hama, H	Biochim Biophys Acta	2010
22517924	Stereospecificity of fatty acid 2-hydroxylase and differential functions of 2-hydroxy fatty acid enantiomers Guo, L, Zhang, X, Zhou, D, Okunade, AL, Su, X	J Lipid Res	2012
36902339	Fatty Acid 2-Hydroxylase and 2-Hydroxylated Sphingolipids: Metabolism and Function in Health and Diseases Eckhardt, M	Int J Mol Sci	2023
17355976	Fatty acid 2-hydroxylase, encoded by FA2H, accounts for differentiation-associated increase in 2-OH ceramides during keratinocyte differentiation Uchida, Y, Hama, H, Alderson, NL, Douangpanya, S, Wang, Y, Crumrine, DA, Elias, PM, Holleran, WM	J. Biol. Chem.	2007
15337768	The human FA2H gene encodes a fatty acid 2-hydroxylase Alderson, NL, Rembiesa, BM, Walla, MD, Bielawska, A, Bielawski, J, Hama, H	J. Biol. Chem.	2004