Literature summary for 2.8.3.5 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.

Search for more literature for 2.8.3.5

Cloned(Commentary)

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

Protein Variants

Protein Variants	Comment	Organism
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)

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

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	gene SCOT	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
fibroblast	-	Homo sapiens	-

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Synonyms

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

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Release 2023.1 (January 2023)