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Lookup NU author(s): Dr Morteza Pourfarzam,
Professor Kim Bartlett
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The trifunctional enzyme comprises three consecutive steps in the mitochondrial beta-oxidation of long-chain acyl-CoA esters: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-ketoacyl-CoA thiolase. Deficiencies in either 3-hydroxyacyl-CoA dehydrogenase activity, or all three activities, are important causes of human disease. The dehydrogenase and thiolase have a requirement for NAD(+) and CoA respectively, whose levels are conserved within the mitochondrion and thus provide possible means for control and regulation of beta-oxidation. Using analysis of the intact CoA ester intermediates produced by the complex, we have examined the sensitivity of the complex to NAD(+)/NADH and acetyl-CoA. We consider the evidence for channelling within the trifunctional protein and propose a model for a beta-oxidation 'metabolon'.
Author(s): Bartlett K; Pourfarzam M; Eaton S; Bursby T; Middleton B; Mills K; Johnson AW
Publication type: Article
Publication status: Published
Journal: Biochemical Society Transactions
ISSN (print): 0300-5127
ISSN (electronic): 1470-8752
Publisher: Portland Press