α-Hydroxylation of lignoceroyl-coa in rat brain microsomes: Involvement of nadph-cytochrome c reductase and topical distribution

Abstract2-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 α-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 hFAsphingolipids in other organs remains largely unknown. Recent evidence indicates that hFAsphingolipids have specific roles in cell signaling.

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A new 6-hydroxy-4-sphingenine-containing ceramide in human skin

Stewart

Downing

1999

Journal of Lipid Research

103

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A new ceramide consisting of 6-hydroxysphingosine linked to a non-hydroxyacid was found in human epidermal lipid. This ceramide was sought because its fatty acid and sphingoid moieties are present in other combinations in human epidermal ceramides. To isolate the new ceramide, the mixture of ceramides in human epidermal lipid was first separated into fractions by thin-layer chromatography (TLC), and then each fraction was further purified by TLC after acetylation of all hydroxyl groups. TLC after acetylation revealed that one of the fractions isolated in the first TLC step contained two components, namely, the ceramide consisting of sphingosine linked to an ␣hydroxyacid and an unknown ceramide. The new ceramide constituted about 9% of the total ceramides, and was shown by NMR spectroscopy to be N-acyl-6-hydroxysphingosine. -Stewart, M. E., and D. T. Downing. A new 6-hydroxy-4-sphingenine-containing ceramide in human skin.

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α-Hydroxylation of lignoceroyl-coa in rat brain microsomes: Involvement of nadph-cytochrome c reductase and topical distribution

Cited by 6 publications

References 18 publications

Mouse Models of FA2H Deficiency

Mouse Models of FA2H Deficiency

Fatty acid 2-Hydroxylation in mammalian sphingolipid biology

A new 6-hydroxy-4-sphingenine-containing ceramide in human skin

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