EC Number | Application | Comment | Organism |
---|---|---|---|
2.8.3.5 | medicine | Succinyl-CoA:3-ketoacid CoA transferase deficiency causes episodic ketoacidosis | Homo sapiens |
EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
2.8.3.5 | R268H | DNA mutation in a south african human with SCOT deficiency, detected in fibroblasts | Homo sapiens |
EC Number | General Stability | Organism |
---|---|---|
2.8.3.5 | An analysis of the three-dimensional structure of SCOT shows that the R268H mutation is expected to break a conserved salt bridge between R268 and D52, which would be expected to lead to decreased stability of the protein. | Homo sapiens |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
2.8.3.5 | mitochondrion | the enzyme is a mitochondrial homodimer | Homo sapiens | 5739 | - |
EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
2.8.3.5 | succinyl-CoA + 3-hydroxybutyrate | Homo sapiens | - |
succinate + 3-hydroxybutyryl-CoA | - |
? | |
2.8.3.5 | succinyl-CoA + a 3-oxo acid | Homo sapiens | ketone bodies, produced mainly in the liver, are an important source of energy for extrahepatic tissues | succinate + a 3-oxoacyl-CoA | - |
? | |
2.8.3.5 | succinyl-CoA + acetoacetate | Homo sapiens | - |
succinate + acetoacetyl-CoA | - |
r |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
2.8.3.5 | Homo sapiens | - |
- |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
2.8.3.5 | fibroblast | of mutant enzyme R268H | Homo sapiens | - |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
2.8.3.5 | succinyl-CoA + 3-hydroxybutyrate | - |
Homo sapiens | succinate + 3-hydroxybutyryl-CoA | - |
? | |
2.8.3.5 | succinyl-CoA + a 3-oxo acid | - |
Homo sapiens | succinate + a 3-oxoacyl-CoA | - |
? | |
2.8.3.5 | succinyl-CoA + a 3-oxo acid | ketone bodies, produced mainly in the liver, are an important source of energy for extrahepatic tissues | Homo sapiens | succinate + a 3-oxoacyl-CoA | - |
? | |
2.8.3.5 | succinyl-CoA + acetoacetate | - |
Homo sapiens | succinate + acetoacetyl-CoA | - |
r |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
2.8.3.5 | SCOT | - |
Homo sapiens |
2.8.3.5 | succinyl-CoA:3-ketoacid coenzyme A transferase | - |
Homo sapiens |
EC Number | Temperature Minimum [°C] | Temperature Maximum [°C] | Comment | Organism |
---|---|---|---|---|
2.8.3.5 | 30 | 40 | 100% SCOT activity of wild-type enzyme | Homo sapiens |
2.8.3.5 | 30 | - |
59.7% residual SCOT activity of R268H mutant enzyme | Homo sapiens |
2.8.3.5 | 37 | - |
34% residual SCOT activity of R268H mutant enzyme | Homo sapiens |
2.8.3.5 | 40 | - |
4% residual SCOT activity of R268H mutant enzyme, the R268H mutant protein has temperature-sensitive instability and dramatically reduces residual SCOT activity to 4% wild-type in 40°C expression so that the R268H mutation is a disease-causing mutation in GS10 and GS11 a SCOT-deficient sibling case from South Africa | Homo sapiens |