InterPro domain: IPR045025

This entry represents a group of TPP-binding domain containing proteins, including oxalyl-CoA decarboxylase from E. coli, IorA from Thermococcus kodakarensis and HACL1 from animals. In general, they are thiamine pyrophosphate-dependent enzymes that catalyse the carbon-carbon bond cleavage [ 1 ]. In humans, HACL1 is a peroxisomal 2-OH acyl-CoA lyase involved in the cleavage (C1 removal) reaction in the fatty acid alpha-oxidation in a thiamine pyrophosphate (TPP)-dependent manner [ 2 , 2 , 3 ]. It is also involved in the degradation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids [ 4 , 4 , 4 ].

1. Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids. Proc Natl Acad Sci U S A 96, 10039-44
2. Phytosphingosine degradation pathway includes fatty acid α-oxidation reactions in the endoplasmic reticulum. Proc Natl Acad Sci U S A 114, E2616-E2623
3. Role of thiamine pyrophosphate in oligomerisation, functioning and import of peroxisomal 2-hydroxyacyl-CoA lyase. Biochim Biophys Acta 1814, 1226-33