2-hydroxyacyl-CoA lyase 1; 2-hydroxyphytanoyl-CoA lyase; 2-HPCL; phytanoyl-CoA 2-hydroxylase 2 (HACL1, HPCL, HPCL2, PHYH2, HSPC279)

Function

• catalyzes a carbon-carbon cleavage reaction
• cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA & a 2-methyl-branched fatty aldehyde
• alpha-oxidation of 3-methyl branched fatty acids (phytanic acid); 3rd step

Cofactor:

• binds 1 Mg+2 per subunit
• binds 1 thiamine pyrophosphate per subunit

Structure

• homotetramer
• belongs to the TPP enzyme family

Compartment

peroxisome

Expression

• expressed in high levels in liver
• also expressed in kidney, heart & skeletal muscle

More general terms

• lyase
• oligomerizing protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=26061
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:26061
• OMIM: https://mirror.omim.org/entry/604300
• UniProt: http://www.uniprot.org/uniprot/Q9UJ83.html