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The mitochondrial trifunctional protein: centre of a beta-oxidation metabolon?

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'.

Publication metadata

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

Year: 2000

Volume: 28

Issue: 2

Pages: 177-182

ISSN (print): 0300-5127

ISSN (electronic): 1470-8752

Publisher: Portland Press