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Literature summary extracted from

  • Gibson, K.M.; Lee, C.F.; Kamali, V.; Soevik, O.
    A coupled assay detecting defects in fibroblasts isoleucine degradation distal to enoyl-CoA hydratase: application to 3-oxothiolase deficiency (1992), Clin. Chim. Acta, 205, 127-135.
    View publication on PubMed

Application

EC Number Application Comment Organism
1.1.1.178 medicine used for coupled enzyme assay to detect 3-oxothiolase deficiency in L-isoleucine degrading pathway Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.1.1.178 2-methyl-3-hydroxybutyryl-CoA + NAD+ Homo sapiens isoleucine metabolism 2-methylacetoacetyl-CoA + NADH
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Organism

EC Number Organism UniProt Comment Textmining
1.1.1.178 Homo sapiens
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-
-

Source Tissue

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

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.1.178 2-methyl-3-hydroxybutyryl-CoA + NAD+
-
Homo sapiens 2-methylacetoacetyl-CoA + NADH
-
?
1.1.1.178 2-methyl-3-hydroxybutyryl-CoA + NAD+ isoleucine metabolism Homo sapiens 2-methylacetoacetyl-CoA + NADH
-
?