FA2H hydroxylates 1,2-saturated fatty acids

Stable Identifier
Reaction [transition]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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

Zhang, X, Guo, L, Zhou, D, Su, X, Okunade, AL

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

Holleran, WM, Hama, H, Alderson, NL, Crumrine, DA, Wang, Y, Elias, PM, Douangpanya, S, Uchida, Y

J. Biol. Chem. 2007
15337768 The human FA2H gene encodes a fatty acid 2-hydroxylase

Hama, H, Alderson, NL, Bielawski, J, Walla, MD, Rembiesa, BM, Bielawska, A

J. Biol. Chem. 2004
Catalyst Activity

fatty acid alpha-hydroxylase activity of FA2H [endoplasmic reticulum membrane]

Orthologous Events
Cite Us!