Thiamine pyrophosphate: an essential cofactor in the mammalian metabolism of 3-methyl-branched fatty acids--implications for thiamine deficiencies?

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A major breakthrough in the research on the alpha-oxidation of 3-methyl-branched fatty acids, such as the naturally occurring phytanic acid (3,7,11,15-tetramethylhexadecanoic acid), was the identification of 2- hydroxyphytanoyl-CoA lyase (2-HPCL), a peroxisomal enzyme that catalyzes the carbon-carbon bond cleavage in the third step of the proposed pathway, and the observation that its activity depends on thiamine pyrophosphate (TPP), a hitherto unknown cofactor of alpha-oxidation (Foulons et al., 1999).