2-hydroxy Lignoceric acid is an α-hydroxy very long chain fatty acid that is normally present in the mammalian nervous system. In the brain, 2-hydroxy lignoceric acid is derived from lignoceric acid and further converted to ceramides and cerebrosides during the process of nerve sheath myelination. 2-hydroxy Lignoceric acid is produced by the α-oxidation of lignoceric acid in the peroxisome and defects in this pathway are associated with disorders such as Zellweger syndrome.