Literature summary for 2.8.3.5 extracted from

Berry, G.T.; Fukao, T.; Mitchell, G.A.; Mazur, A.; Ciafre, M.; Gibson, J.; Kondo, N.; Palmieri, M.J.
Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency (2001), J. Inherit. Metab. Dis., 24, 587-595.

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Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
succinyl-CoA + acetoacetate	Homo sapiens	enzyme deficiency leads to ketoacidotic crises and persistent ketosis	?	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	SCOT-deficient patients	-

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
0.0005	-	fibroblast extract, patient with enzyme deficiency	Homo sapiens

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
succinyl-CoA + acetoacetate	enzyme deficiency leads to ketoacidotic crises and persistent ketosis	Homo sapiens	?	-	?

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Release 2023.1 (January 2023)