The trifunctional enzyme comprises three consecutive steps in the mitochondrial β-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 β-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 β-oxidation ‘metabolon’.
The mitochondrial trifunctional protein: centre of a β-oxidation metabolon?
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S. Eaton, T. Bursby, B. Middleton, M. Pourfarzam, K. Mills, A. W. Johnson, K. Bartlecc; The mitochondrial trifunctional protein: centre of a β-oxidation metabolon?. Biochem Soc Trans 1 February 2000; 28 (2): 177–182. doi: https://doi.org/10.1042/bst0280177
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