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2-hydroxyacyl-CoA lyase 1; 2-hydroxyphytanoyl-CoA lyase; 2-HPCL; phytanoyl-CoA 2-hydroxylase 2 (HACL1, HPCL, HPCL2, PHYH2, HSPC279)
Function:
1) catalyzes a carbon-carbon cleavage reaction
2) cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA & a 2-methyl-branched fatty aldehyde
3) 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
General
lyase
oligomerizing protein
phosphoprotein
Properties
SIZE: entity length = 578 aa
MW = 64 kD
COMPARTMENT: peroxisome
MOTIF: Ser phosphorylation site {S4}
binding site
SITE: 60-60
FOR-BINDING-OF: Thiamine pyrophosphate
Thiamine pyrophosphate binding {401-486}
MOTIF: Mg+2-binding site
SITE: 455-455
Mg+2-binding site
SITE: 482-482
microbody-targeting signal {576-578}
Database Correlations
OMIM 604300
UniProt Q9UJ83
PFAM correlations
Entrez Gene 26061
Kegg hsa:26061
References
UniProt :accession Q9UJ83