...C.J. Schofield; M.A. McDonough Phytanic acid (PA) is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric β-methyl group, PA cannot be metabolized by β-oxidation. Instead, it is metabolized in peroxisomes via α-oxidation to give pristanic acid...

...). It was discovered in 1999, when studying α-oxidation of phytanic acid. HACL1 has an important role in at least two pathways: (i) the degradation of 3-methyl-branched fatty acids like phytanic acid and (ii) the shortening of 2-hydroxy long-chain fatty acids. In both cases, HACL1 catalyses the cleavage step, which...

... of phytanic acid have led to the clarification of the pathophysiology of Refsum's disease, rhizomelic chondrodysplasia and AMACR (α-methylacyl-CoA racemase) deficiency. This has highlighted the role of an Fe(II)- and 2-oxoglutarate-dependent oxygenases [PhyH (phytanoyl-CoA 2-hydroxylase), also known as PAHX...

...A.S. Wierzbicki; M.D. Lloyd Branched-chain fatty acids are common components of the human diet (phytanic acid) or are produced endogenously (bile acids), and are also used as medicines (ibuprofen). Owing to their branched-chain structure, they are metabolized in peroxisomes. In the case of phytanic...

...R.J.A. Wanders; J.C. Komen In the present paper, we describe the current state of knowledge regarding the enzymology of the phytanic acid α-oxidation pathway. The product of phytanic acid α-oxidation, i.e. pristanic acid, undergoes three cycles of β-oxidation in peroxisomes after which the products...

...-hydroxyacyl-CoA, generating formyl-CoA (precursor of formate/CO 2 ) and, in case of phytanic acid as substrate, pristanal (precursor of pristanic acid). The stereochemistry of the overall pathway, cofactor requirements and substrate specificity of the hydroxylase and the cleavage enzyme, which is homologous...