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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. 2007 Nov; 35(Pt 5):876-80.BS

Abstract

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 alpha-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 CO(2). 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.

Authors+Show Affiliations

Department of Molecular Cell Biology, Division of Pharmacology, LIPIT, K.U. Leuven, O&N1, Herestraat 49, Box 601, B-3000 Leuven, Belgium. minne.casteels@med.kuleuven.beNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

17956236

Citation

Casteels, M, et al. "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." Biochemical Society Transactions, vol. 35, no. Pt 5, 2007, pp. 876-80.
Casteels M, Sniekers M, Fraccascia P, et al. 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. 2007;35(Pt 5):876-80.
Casteels, M., Sniekers, M., Fraccascia, P., Mannaerts, G. P., & Van Veldhoven, P. P. (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. Biochemical Society Transactions, 35(Pt 5), 876-80.
Casteels M, et al. 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. 2007;35(Pt 5):876-80. PubMed PMID: 17956236.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Casteels,M, AU - Sniekers,M, AU - Fraccascia,P, AU - Mannaerts,G P, AU - Van Veldhoven,P P, PY - 2007/10/25/pubmed PY - 2008/2/6/medline PY - 2007/10/25/entrez SP - 876 EP - 80 JF - Biochemical Society transactions JO - Biochem Soc Trans VL - 35 IS - Pt 5 N2 - 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 alpha-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 CO(2). 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. SN - 0300-5127 UR - https://www.unboundmedicine.com/medline/citation/17956236/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_ L2 - https://portlandpress.com/biochemsoctrans/article-lookup/doi/10.1042/BST0350876 DB - PRIME DP - Unbound Medicine ER -