HACL1

HACL1 (2-Hydroxyacyl-CoA Lyase 1) is a Protein Coding gene. Diseases associated with HACL1 include Alpha-Methylacyl-Coa Racemase Deficiency and Beriberi. Among its related pathways are Peroxisomal lipid metabolism and Metabolism. Gene Ontology (GO) annotations related to this gene include identical protein binding and magnesium ion binding. An important paralog of this gene is ILVBL.

Full Name

2-Hydroxyacyl-CoA Lyase 1

Function

Peroxisomal 2-OH acyl-CoA lyase involved in the cleavage (C1 removal) reaction in the fatty acid alpha-oxydation in a thiamine pyrophosphate (TPP)-dependent manner (PubMed:28289220, PubMed:21708296, PubMed:10468558).

Involved in the degradation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids (PubMed:28289220, PubMed:21708296, PubMed:10468558).

Plays a significant role in the biosynthesis of heptadecanal in the liver (By similarity).

Biological Process

Fatty acid alpha-oxidation Source: UniProtKB
Fatty acid metabolic process Source: UniProtKB
Methyl-branched fatty acid metabolic process Source: UniProtKB
Phytanic acid metabolic process Source: UniProtKB
Protein targeting to peroxisome Source: UniProtKB

Cellular Location

Peroxisome