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HACL1

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:10468558, PubMed:21708296, PubMed:28289220). Involved in the degradation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids (PubMed:10468558, PubMed:21708296, PubMed:28289220). Plays a significant role in the biosynthesis of heptadecanal in the liver (By similarity).

Pathway

Lipid metabolism; fatty acid metabolism.

Sequence Similarities

Belongs to the TPP enzyme family.

Tissue Specificity

Widely expressed.

Cellular localization

Alternative names

HPCL, HPCL2, PHYH2, HSPC279, HACL1, 2-hydroxyacyl-CoA lyase 1, 2-hydroxyphytanoyl-CoA lyase, Phytanoyl-CoA 2-hydroxylase 2, 2-HPCL

swissprot:Q9UJ83 entrezGene:26061 omim:604300