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Literature summary extracted from

  • Fukao, T.; Kursula, P.; Owen, E.P.; Kondo, N.
    Identification and characterization of a temperature-sensitive R268H mutation in the human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene (2007), Mol. Genet. Metab., 92, 216-221.
    View publication on PubMed

Application

EC Number Application Comment Organism
2.8.3.5 medicine Succinyl-CoA:3-ketoacid CoA transferase deficiency causes episodic ketoacidosis Homo sapiens

Protein Variants

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

General Stability

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

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.8.3.5 mitochondrion the enzyme is a mitochondrial homodimer Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

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

Organism

EC Number Organism UniProt Comment Textmining
2.8.3.5 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.8.3.5 fibroblast of mutant enzyme R268H Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

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

Temperature Range [°C]

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