HACL1


Description

The HACL1 (2-hydroxyacyl-CoA lyase 1) is a protein-coding gene located on chromosome 3.

HACL1 is a peroxisomal enzyme that plays a crucial role in the alpha-oxidation of fatty acids. It acts as a 2-hydroxyacyl-CoA lyase, catalyzing the cleavage of the first carbon atom (C1 removal) from 2-hydroxy fatty acyl-CoA substrates. This process is dependent on thiamine pyrophosphate (TPP). HACL1 is involved in the degradation of 3-methyl-branched fatty acids, such as phytanic acid, and the shortening of 2-hydroxy long-chain fatty acids. Moreover, it contributes significantly to the biosynthesis of heptadecanal in the liver. These functions are supported by evidence from UniProtKB (Q9QXE0) and PubMed publications (10468558, 21708296, 28289220).

HACL1 is also known as 2-HPCL, HPCL, HPCL2, PHYH2.

Associated Diseases


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