title: Alpha-oxidation of 3-methyl-substituted fatty acids and its thiamine dependence.
abstract: 3-Methyl-branched fatty acids, as phytanic acid, undergo peroxisomal alpha-oxidation in which they are shortened by 1 carbon atom. This process includes four steps: activation, 2-hydroxylation, thiamine pyrophosphate dependent cleavage and aldehyde dehydrogenation. The thiamine pyrophosphate dependence of the third step is unique in peroxisomal mammalian enzymology. Human pathology due to a deficient alpha-oxidation is mostly linked to mutations in the gene coding for the second enzyme of the sequence, phytanoyl-CoA hydroxylase.
authors.affiliation: Afdeling Farmacologie, Department of Molecular Cell Biology, Katholieke Universiteit Leuven, Belgium. minne.casteels@med.kuleuven.ac.be
authors.forename: Minne
authors.initials: M
authors.lastname: Casteels
authors.forename: Veerle
authors.initials: V
authors.lastname: Foulon
authors.forename: Guy P
authors.initials: GP
authors.lastname: Mannaerts
authors.forename: Paul P
authors.initials: PP
authors.lastname: Van Veldhoven
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D008745:Methylation; D006899:Mixed Function Oxygenases / Q000737:chemistry / Q000235:genetics; D008969:Molecular Sequence Data; D010084:Oxidation-Reduction; D010831:Phytanic Acid / Q000378:metabolism; D016415:Sequence Alignment; D017386:Sequence Homology, Amino Acid; D013237:Stereoisomerism; D013831:Thiamine / Q000494:pharmacology*
chemical_list: D005227:Fatty Acids; D010831:Phytanic Acid; D006899:Mixed Function Oxygenases; C102623:PHYH protein, human; D013831:Thiamine