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Literature summary for 1.3.8.6 extracted from

  • Sauer, S.W.
    Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency (2007), J. Inherit. Metab. Dis., 30, 673-680.
    View publication on PubMed

Application

Application Comment Organism
medicine glutaryl-CoA dehydrogenase deficiency is an autosomal recessive disease characterized by the accumulation of glutaric and 3-hydroxyglutaric acids in tissues and body fluids causing uncompetitive inhibition of alpha-ketoglutarate dehydrogenase complex Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens GCDH is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
glutaryl-CoA + acceptor
-
Homo sapiens crotonyl-CoA + CO2 + reduced acceptor
-
?
additional information GCDH is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine Homo sapiens ?
-
?

Synonyms

Synonyms Comment Organism
GCDH
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
FAD
-
Homo sapiens