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3-Methyl-branched fatty acids, as phytanic acid, undergoperoxisomala-oxidation in which they are shortened by 1carbon atom. This process includes four steps: activation,2-hydroxylation, thiamine pyrophosphate dependentcleavage and aldehyde dehydrogenation. The thiaminepyrophosphate dependence of the third step is unique inperoxisomal mammalian enzymology. Human pathologydue to a deficient alpha-oxidation is mostly linked tomutations in the gene coding for the second enzyme of thesequence, phytanoyl-CoA hydroxylase....
Nội dung trích xuất từ tài liệu:
Báo cáo khoa học: Alpha-oxidation of 3-methyl-substituted fatty acids and its thiamine dependence
