Literature summary extracted from

Koelker, S.; Christensen, E.; Leonard, J.V.; Greenberg, C.R.; Burlina, A.B.; Burlina, A.P.; Dixon, M.; Duran, M.; Goodman, S.I.; Koeller, D.M.; Mueller, E.; Naughten, E.R.; Neumaier-Probst, E.; Okun, J.G.; Kyllerman, M.; Surtees, R.A.; Wilcken, B.; Hoffmann, G.F.; Burgard, P.
Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I) (2007), J. Inherit. Metab. Dis., 30, 5-22.

Application

EC Number	Application	Comment	Organism
1.3.8.6	medicine	glutaryl-CoA dehydrogenase deficiency (glutaric acidaemia or aciduria type I) is an autosomal recessive disease which is characterized by an accumulation of glutaric acid, 3-hydroxyglutaric acid, glutaconic acid (less frequently), and glutarylcarnitine in body fluids and tissues	Homo sapiens

Localization

EC Number	Localization	Comment	Organism	GeneOntology No.	Textmining
1.3.8.6	mitochondrion	-	Homo sapiens	5739	-

Organism

EC Number	Organism	UniProt	Comment	Textmining
1.3.8.6	Homo sapiens	-	-	-

Source Tissue

EC Number	Source Tissue	Comment	Organism	Textmining
1.3.8.6	fibroblast	-	Homo sapiens	-
1.3.8.6	leukocyte	-	Homo sapiens	-

Substrates and Products (Substrate)

EC Number	Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
1.3.8.6	glutaryl-CoA + acceptor	-	Homo sapiens	crotonyl-CoA + CO2 + reduced acceptor	-	?

Synonyms

EC Number	Synonyms	Comment	Organism
1.3.8.6	GCDH	-	Homo sapiens

Cofactor

EC Number	Cofactor	Comment	Organism	Structure
1.3.8.6	FAD	dependent	Homo sapiens

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Release 2023.1 (January 2023)