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Carnitine acyltransferases and their influence on CoA pools in health and disease.
Mol Aspects Med. 2004 Oct-Dec; 25(5-6):475-93.MA

Abstract

Cells contain limited and sequestered pools of Coenzyme A (CoA) that are essential for activating carboxylate metabolites. Some acyl-CoA esters have high metabolic and signalling impact, so control of CoA ester concentrations is important. This and transfer of the activated acyl moieties between cell compartments without wasting energy on futile cycles of hydrolysis and resynthesis is achieved through the carnitine system. The location, properties of and deficiencies in the carnitine acyltransferases are described in relation to their influence on the CoA pools in the cell and, hence, on metabolism. The protection of free CoA pools in disease states is achieved by excretion of acyl-carnitine so that carnitine supplementation is required where unwanted acyl groups build up, such as in some inherited disorders of fatty acid oxidation. Acetyl-carnitine improves cognition in the brain and propionyl-carnitine improves cardiac performance in heart disease and diabetes. The therapeutic effects of carnitine and its esters are discussed in relation to the integrative influence of the carnitine system across CoA pools. Recent evidence for sequestered pools of activated acetate for synthesis of malonyl-CoA, for the synthesis of polyunsaturated fatty acids and for the inhibition of carnitine palmitoyltransferase 1 to regulate fatty acid oxidation is reviewed.

Authors+Show Affiliations

Centre for Biomolecular Sciences, University of St. Andrews, North Haugh, St. Andrews KY16 9ST, Scotland, UK. rrr@st-and.ac.ukNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

15363637

Citation

Ramsay, Rona R., and Victor A. Zammit. "Carnitine Acyltransferases and Their Influence On CoA Pools in Health and Disease." Molecular Aspects of Medicine, vol. 25, no. 5-6, 2004, pp. 475-93.
Ramsay RR, Zammit VA. Carnitine acyltransferases and their influence on CoA pools in health and disease. Mol Aspects Med. 2004;25(5-6):475-93.
Ramsay, R. R., & Zammit, V. A. (2004). Carnitine acyltransferases and their influence on CoA pools in health and disease. Molecular Aspects of Medicine, 25(5-6), 475-93.
Ramsay RR, Zammit VA. Carnitine Acyltransferases and Their Influence On CoA Pools in Health and Disease. Mol Aspects Med. 2004 Oct-Dec;25(5-6):475-93. PubMed PMID: 15363637.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Carnitine acyltransferases and their influence on CoA pools in health and disease. AU - Ramsay,Rona R, AU - Zammit,Victor A, PY - 2004/9/15/pubmed PY - 2005/10/29/medline PY - 2004/9/15/entrez SP - 475 EP - 93 JF - Molecular aspects of medicine JO - Mol Aspects Med VL - 25 IS - 5-6 N2 - Cells contain limited and sequestered pools of Coenzyme A (CoA) that are essential for activating carboxylate metabolites. Some acyl-CoA esters have high metabolic and signalling impact, so control of CoA ester concentrations is important. This and transfer of the activated acyl moieties between cell compartments without wasting energy on futile cycles of hydrolysis and resynthesis is achieved through the carnitine system. The location, properties of and deficiencies in the carnitine acyltransferases are described in relation to their influence on the CoA pools in the cell and, hence, on metabolism. The protection of free CoA pools in disease states is achieved by excretion of acyl-carnitine so that carnitine supplementation is required where unwanted acyl groups build up, such as in some inherited disorders of fatty acid oxidation. Acetyl-carnitine improves cognition in the brain and propionyl-carnitine improves cardiac performance in heart disease and diabetes. The therapeutic effects of carnitine and its esters are discussed in relation to the integrative influence of the carnitine system across CoA pools. Recent evidence for sequestered pools of activated acetate for synthesis of malonyl-CoA, for the synthesis of polyunsaturated fatty acids and for the inhibition of carnitine palmitoyltransferase 1 to regulate fatty acid oxidation is reviewed. SN - 0098-2997 UR - https://www.unboundmedicine.com/medline/citation/15363637/Carnitine_acyltransferases_and_their_influence_on_CoA_pools_in_health_and_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0098299704000470 DB - PRIME DP - Unbound Medicine ER -