ExplorEnz: EC 4.1.2.63

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4.1.2.63

Accepted name:

2-hydroxyacyl-CoA lyase

Reaction:

(1) a 2-hydroxy-3-methyl-C_n-fatty-acyl-CoA = a 2-methyl-branched C_n-1-fatty aldehyde + formyl-CoA
(2) a (2R)-2-hydroxy-C_n-long-chain fatty acyl-CoA = a C_n-1-long-chain fatty aldehyde + formyl-CoA

Other name(s):

HACL1 (gene name); 2-hydroxyphytanoyl-CoA lyase; 2-HPCL

Systematic name:

2-hydroxy-3-methyl fatty-CoA formyl-CoA lyase (2-methyl branched fatty aldehyde-forming)

Comments:

Requires Mg²⁺ and thiamine diphosphate. This peroxisomal enzyme, found in animals, is involved in the α-oxidation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids.

Links to other databases:

BRENDA, EXPASY, Gene, KEGG, MetaCyc, PDB

References:

1.	Foulon, V., Antonenkov, V.D., Croes, K., Waelkens, E., Mannaerts, G.P., Van Veldhoven, P.P. and Casteels, M. Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during α-oxidation of 3-methyl-branched fatty acids. Proc. Natl. Acad. Sci. USA 96 (1999) 10039–10044. [DOI] [PMID: 10468558]
2.	Foulon, V., Sniekers, M., Huysmans, E., Asselberghs, S., Mahieu, V., Mannaerts, G.P., Van Veldhoven, P.P. and Casteels, M. Breakdown of 2-hydroxylated straight chain fatty acids via peroxisomal 2-hydroxyphytanoyl-CoA lyase: a revised pathway for the α-oxidation of straight chain fatty acids. J. Biol. Chem. 280 (2005) 9802–9812. [DOI] [PMID: 15644336]
3.	Casteels, M., Sniekers, M., Fraccascia, P., Mannaerts, G.P. and Van Veldhoven, P.P. The role of 2-hydroxyacyl-CoA lyase, a thiamin pyrophosphate-dependent enzyme, in the peroxisomal metabolism of 3-methyl-branched fatty acids and 2-hydroxy straight-chain fatty acids. Biochem Soc Trans. 35 (2007) 876–880. [DOI] [PMID: 17956236]

[EC 4.1.2.63 created 2021]