Literature summary for 1.1.1.178 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

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-

Substrates and Products (Substrate)

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