title: The chemical biology of branched-chain lipid metabolism.
abstract: Mammalian metabolism of some lipids including 3-methyl and 2-methyl branched-chain fatty acids occurs within peroxisomes. Such lipids, including phytanic and pristanic acids, are commonly found within the human diet and may be derived from chlorophyll in plant extracts. Due to the presence of a methyl group at its beta-carbon, the well-characterised beta-oxidation pathway cannot degrade phytanic acid. Instead its alpha-methylene group is oxidatively excised to give pristanic acid, which can be metabolised by the beta-oxidation pathway. Many defects in the alpha-oxidation pathway result in an accumulation of phytanic acid, leading to neurological distress, deterioration of vision, deafness, loss of coordination and eventual death. Details of the alpha-oxidation pathway have only recently been elucidated, and considerable progress has been made in understanding the detailed enzymology of one of the oxidative steps within this pathway. This review summarises these recent advances and considers the roles and likely mechanisms of the enzymes within the alpha-oxidation pathway.
authors.affiliation: The Oxford Centre for Molecular Sciences & The Dyson Perrins Laboratory, South Parks Road, Oxford OX1 3QY, UK.
authors.forename: Mridul
authors.initials: M
authors.lastname: Mukherji
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Schofield
authors.forename: Anthony S
authors.initials: AS
authors.lastname: Wierzbicki
authors.forename: Gerbert A
authors.initials: GA
authors.lastname: Jansen
authors.forename: Ronald J A
authors.initials: RJ
authors.lastname: Wanders
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Lloyd
mesh_terms: D005227:Fatty Acids / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D010084:Oxidation-Reduction; D018901:Peroxisomal Disorders / Q000378:metabolism*; D020675:Peroxisomes / Q000378:metabolism; D010831:Phytanic Acid / Q000378:metabolism
chemical_list: D005227:Fatty Acids; D010831:Phytanic Acid