| Literature DB >> 12694175 |
Minne Casteels1, Veerle Foulon, Guy P Mannaerts, Paul P Van Veldhoven.
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.Entities:
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Year: 2003 PMID： 12694175 DOI： 10.1046/j.1432-1033.2003.03534.x
Source DB: PubMed Journal: Eur J Biochem ISSN： 0014-2956