alpha-Hydroxy very long chain fatty acids are abundant in nervous tissues and are components of cerebrosides and sulfatides, which are mostly found in myelin of nervous tissues. 2-Hydroxytetracosanoic acid, which is unique to nervous tissues, is formed by the oxidation of tetracosanoic acid by the enzyme fatty acid 2-hydroxylase. This enzyme is also responsible for the formation of 2-hydroxy galactolipids in the peripheral nervous system.1 lpha-Oxidation of 2-hydroxytetracosanoic acid to CO2 and tricosanoic acid occurs in the peroxisome and is unique from the lpha-oxidation of beta-carbon branched fatty acids such as phytanic acid. Cells from Zellweger syndrome and peroxisome-deficient cells are unable to undergo lphaoxidation although patients with other peroxisomal disorders such as X-linked adrenoleukodystrophy, Refsum disease, and rhizomelic chondrodysplasia punctata were able.2 2-Hydroxytetracosanoic acid is undergoing much research and various methods of analysis are being investigated.3
