Article
The mitochondrial trifunctional protein: centre of a beta-oxidation metabolon?
Biochemical Society transactions - 1 Feb 2000
Eaton S, Bursby T, Middleton B, Pourfarzam M, Mills K, Johnson A W, Bartlett K
Abstract excerpt
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...
Topics
- Amino Acid Sequence
- Catalysis
- Humans
- Mitochondrial Trifunctional Protein
- Models, Biological
- Molecular Sequence Data
- Multienzyme Complexes
- Mutation
- Oxygen
- Protein Structure, Tertiary