2-Hydroxyphytanoyl-CoA lyase (abbreviated as 2-HPCL), renamed to 2-hydroxyacyl-CoA lyase (abbreviated as HACL1), is the first peroxisomal enzyme in mammals that has been found to be dependent on TPP (thiamin pyrophosphate). It was discovered in 1999, when studying α-oxidation of phytanic acid. HACL1 has an important role in at least two pathways: (i) the degradation of 3-methyl-branched fatty acids like phytanic acid and (ii) the shortening of 2-hydroxy long-chain fatty acids. In both cases, HACL1 catalyses the cleavage step, which involves the splitting of a carbon–carbon bond between the first and second carbon atom in a 2-hydroxyacyl-CoA intermediate leading to the production of an (n−1) aldehyde and formyl-CoA. The latter is rapidly converted into formate and subsequently to CO2. HACL1 is a homotetramer and has a PTS (peroxisomal targeting signal) at the C-terminal side (PTS1). No deficiency of HACL1 has been described yet in human, but thiamin deficiency might affect its activity.
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November 2007
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Conference Article|
October 25 2007
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
M. Casteels;
M. Casteels
1
1Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium
1To whom correspondence should be addressed (email minne.casteels@med.kuleuven.be).
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M. Sniekers;
M. Sniekers
1Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium
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P. Fraccascia;
P. Fraccascia
1Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium
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G.P. Mannaerts;
G.P. Mannaerts
1Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium
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P.P. Van Veldhoven
P.P. Van Veldhoven
1Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium
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Publisher: Portland Press Ltd
Received:
June 11 2007
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2007 Biochemical Society
2007
Biochem Soc Trans (2007) 35 (5): 876–880.
Article history
Received:
June 11 2007
Citation
M. Casteels, M. Sniekers, P. Fraccascia, G.P. Mannaerts, P.P. Van Veldhoven; 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 1 November 2007; 35 (5): 876–880. doi: https://doi.org/10.1042/BST0350876
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