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ILVBL

Function

Endoplasmic reticulum 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. Involved in the phytosphingosine degradation pathway.

Sequence Similarities

Belongs to the TPP enzyme family.

Tissue Specificity

Expressed in all tissues tested, with highest expression in heart, pancreas and placenta.

Cellular localization

Alternative names

AHAS, HACL2, ILVBL, 2-hydroxyacyl-CoA lyase 2, Acetolactate synthase-like protein, IlvB-like protein

swissprot:A1L0T0 omim:605770 entrezGene:10994