title: Fatty acid 2-Hydroxylation in mammalian sphingolipid biology.
abstract: 2-Hydroxy fatty acids (hFA) are important components of a subset of mammalian sphingolipids. The presence of hFA in sphingolipids is best described in the nervous system, epidermis, and kidney. However, the literature also indicates that various hFA-sphingolipids are present in additional tissues and cell types, as well as in tumors. Biosynthesis of hFA-sphingolipids requires fatty acid 2-hydroyxlase, and degradation of hFA-sphingolipids depends, at least in part, on lysosomal acid ceramidase and the peroxisomal fatty acid alpha-oxidation pathway. Mutations in the fatty acid 2-hydroxylase gene, FA2H, have been associated with leukodystrophy and spastic paraparesis in humans, underscoring the importance of hFA-sphingolipids in the nervous system. In the epidermis, hFA-ceramides are essential for the permeability barrier function. Physiological function of hFA-sphingolipids in other organs remains largely unknown. Recent evidence indicates that hFA-sphingolipids have specific roles in cell signaling.
authors.affiliation: Department of Biochemistry and Molecular Biology, Medical University of South Carolina, Charleston, SC 29425, USA. hama@musc.edu
authors.forename: Hiroko
authors.initials: H
authors.lastname: Hama
mesh_terms: D000818:Animals; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D006900:Hydroxylation; D006899:Mixed Function Oxygenases / Q000378:metabolism*; D013107:Sphingolipids / Q000378:metabolism*
chemical_list: D005227:Fatty Acids; D013107:Sphingolipids; D006899:Mixed Function Oxygenases; C019081:fatty acid alpha-hydroxylase