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Literature summary for 2.8.3.5 extracted from

  • Fukao, T.; Shintaku, H.; Kusubae, R.; Zhang, G.X.; Nakamura, K.; Kondo, M.; Kondo, N.
    Patients homozygous for the T435N mutation of succinyl-CoA:3-ketoacid CoA Transferase (SCOT) do not show permanent ketosis (2004), Pediatr. Res., 56, 858-863.
    View publication on PubMed
Search for more literature for 2.8.3.5

Application

Application Comment Organism
medicine patients homozygous for T435N mutation of SCOT do not show permanent ketosis Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression in SCOT-deficient SV 40-transformed fibroblasts Homo sapiens

Protein Variants

Protein Variants Comment Organism
C456F no activity Homo sapiens
T435N retains significant residual SCOT activities Homo sapiens
V133E no activity Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
succinyl-CoA + acetoacetate Homo sapiens
-
 succinate + acetoacetyl-CoA
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
 mutant T435N shows 20% of wild type activity at 39.5°C, 25% at 37°C, and 50% at 30°C Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
succinyl-CoA + acetoacetate
-
 Homo sapiens succinate + acetoacetyl-CoA
-
 ?

Synonyms

Synonyms Comment Organism
SCOT
-
 Homo sapiens
succinyl-CoA:3-ketoacid CoA transferase
-
 Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
42
-
 incubation of wild type at 42°C for 10 min does not affect enzyme activity, incubation of mutant T435N at 42°C for 10 min reduces enzyme activity to 50% Homo sapiens
55
-
 incubation of wild type at 55°C for 10 min reduces enzyme activity to 20%, incubation of T435N at 55°C results in more rapid inactivation of enzyme activity (below 10% in a 4-min incubation) Homo sapiens