Application Notes:
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 alpha-Oxidation of 2-hydroxytetracosanoic acid to
CO2 and tricosanoic acid occurs in the peroxisome and is unique from the alpha-oxidation of beta-carbon branched fatty
acids such as phytanic acid. Cells from Zellweger syndrome and peroxisome-deficient cells are unable to undergo alphaoxidation
although patients with other peroxisomal disorders such as X-linked adrenoleukodystrophy, Refsum disease, and
rhizomelic chondrodysplasia punctata were able to.2 2-Hydroxytetracosanoic acid is undergoing much research and various
methods of analysis are being investigated.3
References:
1. E. Maldonado et al. “FA2H is responsible for the formation of 2-hydroxy galactolipids in peripheral nervous system myelin” Journal of Lipid Research, Vol. 49 pp. 153-161, 2008
2. R. Sandhir, M. Khan, and I. Singh “Identification of the Pathway of alpha -Oxidation of Cerebronic Acid in Peroxisomes” Lipids, Vol. 35(10) pp. 1127-1133, 2000
3. N. Alderson, M.Walla, and H. Hama “A novel method for the measurement of in vitro fatty acid 2-hydroxylase activity by gas chromatography-mass spectrometry” Journal of Lipid Research, Vol. 46 pp. 1569-1579, 2005