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Literature summary extracted from

  • Yamada, K.; Fukao, T.; Zhang, G.; Sakurai, S.; Ruiter, J.P.; Wanders, R.J.; Kondo, N.
    Single-base substitution at the last nucleotide of exon 6 (c.671G>A), resulting in the skipping of exon 6, and exons 6 and 7 in human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene (2007), Mol. Genet. Metab., 90, 291-297.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.8.3.5 gene SCOT, DNA and amino acid sequence determination and analysis, mutant sequence analysis, overview Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
2.8.3.5 additional information identification of point mutation leading to enzyme inactivation, and deficiency causing severe ketoacidosis in vivo, some mutations lead to highly reduced mRNA and enzyme levels, overview Homo sapiens

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 + acetoacetate Homo sapiens key enzyme for ketone body utilization, SCOT deficiency causes episodes of severe ketoacidosis succinate + acetoacetyl-CoA
-
 r

Organism

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

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.8.3.5 fibroblast
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
2.8.3.5 additional information
-
-
 Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.8.3.5 succinyl-CoA + acetoacetate
-
 Homo sapiens succinate + acetoacetyl-CoA
-
 r
2.8.3.5 succinyl-CoA + acetoacetate key enzyme for ketone body utilization, SCOT deficiency causes episodes of severe ketoacidosis 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 CoA transferase
-
 Homo sapiens