TAILIEUCHUNG - Báo cáo khoa học: Alpha-oxidation of 3-methyl-substituted fatty acids and its thiamine dependence

3-Methyl-branched fatty acids, as phytanic acid, undergo peroxisomala-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 | Eur. J. Biochem. 270 1619-1627 2003 FEBS 2003 doi REVIEW ARTICLE Alpha-oxidation of 3-methyl-substituted fatty acids and its thiamine dependence Minne Casteels Veerle Foulon Guy P. Mannaerts and Paul P. Van Veldhoven Afdeling Farmacologie Department of Molecular Cell Biology Katholieke Universiteit Leuven Belgium 3-Methyl-branched fatty acids as phytanic acid undergo peroxisomal a-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. Keywords alpha-oxidation thiamine pyrophosphate peroxisomes lyase Adult Refsum Disease. Introduction a-Oxidation is the process in which fatty acids are shortened at the carboxyl-end by one carbon atom. For 3-methyl-branched fatty acids this is the preferred pathway as their breakdown by b-oxidation is impossible. Indeed the 3-methyl-branch precludes the third step of b-oxidation the dehydrogenation step. Phytanic acid 3 7 11 15-tetra-methylhexadecanoic acid is at present the only established physiological substrate of a-oxidation in humans 1 2 . Phytanic acid is derived from phytol the isoprenoid side chain of chlorophyll. As chlorophyll-bound phytol cannot be metabolized by humans and free phytol is present only in minimal quantities in food the phytanic acid present in the human body is mostly provided by external sources Fig. 1 . Ruminants ingest large amounts of chlorophyll from which phytol is efficiently cleaved off by bacteria in the gastrointestinal tract. Phytol is subsequently taken up and converted to phytanic acid which is deposited in fat tissues and in milk the major sources of phytanic acid .

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