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 []. 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 [, , ]. 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 [, , ].
