Fatty acid 2-Hydroxylation in mammalian sphingolipid biology

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 barner function Physiological function of hFA-sphingolipids in other organs remains largely unknown Recent evidence indicates that hFA-sphingolipids have specific roles in cell signaling. (C) 2009 Elsevier B V. All rights reserved