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’.
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February 2000
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Conference Article|
February 01 2000
The mitochondrial trifunctional protein: centre of a β-oxidation metabolon?
S. Eaton;
S. Eaton
1
*Unit of Paediatric Surgery, Institute of Child Health, 30 Guilford Street, London WCIN IEH, U.K.
1To whom correspondence should be addressed.
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T. Bursby;
T. Bursby
†Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NEI 4LP, U.K.
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B. Middleton;
B. Middleton
‡Department of Biochemistry, University of Nottingham Medical School, Queens Medical Centre, Nottingham NG7 2UH, U.K.
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M. Pourfarzam;
M. Pourfarzam
†Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NEI 4LP, U.K.
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K. Mills;
K. Mills
*Unit of Paediatric Surgery, Institute of Child Health, 30 Guilford Street, London WCIN IEH, U.K.
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A. W. Johnson;
A. W. Johnson
*Unit of Paediatric Surgery, Institute of Child Health, 30 Guilford Street, London WCIN IEH, U.K.
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K. Bartlecc
K. Bartlecc
†Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NEI 4LP, U.K.
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Publisher: Portland Press Ltd
Received:
August 26 1999
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2000 Biochemical Society
2000
Biochem Soc Trans (2000) 28 (2): 177–182.
Article history
Received:
August 26 1999
Citation
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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