| Predicate | Object |
|---|---|
| rdf:type | |
| biopax3:cellularLocation | |
| biopax3:comment |
The mitochondrial |FRAME: HS01447-MONOMER succinyl-CoA:3-ketoacid-coenzyme A transferase 1| (SCOT) activity is the main determinant of the ketolytic capacity of tissues and is not present in liver, so that organ can not use ketone bodies for fuel, but instead exports them to blood for distribution to peripheral tissues |CITS: [9380443]|. The enzyme is active as a homodimer in the mitochondrial matrix and activates acetoacetate to acetoacetate-CoA for energy production by terminal oxidative phosphorylation. Hereditary SCOT deficiency, including mutations at amino acids 219, 221, 324 and S283X, results in severe episodes of ketoacidosis |CITS: [8751852]||CITS: [10964512]|.
|
| biopax3:xref | |
| biopax3:dataSource | |
| biopax3:displayName |
Scot-S
|
| biopax3:name |
3-oxoacid-CoA transferase 1,
Somatic-type succinyl CoA:3-oxoacid CoA-transferase
|
| biopax3:entityReference | |
| biopax3:standardName |
succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
|