Glutaryl-CoA Dehydrogenase

A flavoprotein enzyme that is responsible for the catabolism of LYSINE; HYDROXYLYSINE; and TRYPTOPHAN. It catalyzes the oxidation of GLUTARYL-CoA to crotonoyl-CoA using FAD as a cofactor. Glutaric aciduria type I is an inborn error of metabolism due to the deficiency of glutaryl-CoA dehydrogenase.

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Networked: 83 relevant articles (2 outcomes, 0 trials/studies)

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Bio-Agent Context: Research Results

Experts

1.	Wajner, Moacir: 22 articles (08/2022 - 06/2007)
2.	Seminotti, Bianca: 12 articles (08/2022 - 09/2012)
3.	Woontner, Michael: 11 articles (01/2022 - 02/2002)
4.	Amaral, Alexandre Umpierrez: 9 articles (12/2020 - 09/2012)
5.	Leipnitz, Guilhian: 8 articles (08/2022 - 08/2015)
6.	Goodman, Stephen: 6 articles (10/2017 - 09/2012)
7.	Koeller, David M: 6 articles (10/2017 - 02/2002)
8.	Ribeiro, Rafael Teixeira: 5 articles (08/2022 - 11/2016)
9.	Vargas, Carmen Regla: 5 articles (01/2021 - 01/2018)
10.	Guerreiro, Gilian: 4 articles (01/2021 - 12/2018)

Related Diseases

1.	Movement Disorders (Movement Disorder) 03/01/2012 - "Concentrations in excess of 300 μM corresponded to dose dependent loss of cell viability which was associated with enhanced production of H(2)O(2) concomitant to elevation of gene expression for diverse antioxidant enzymes; biliverdin reductase, arsenite inducible RNA associated protein, dithiolethione-inducible gene-1 (DIG-1) and thioredoxin reductase 1. Moreover, Mn initiated significant reduction of gene expression of mitochondrial glutaryl-coenzyme A dehydrogenase (GCDH), an enzyme involved with glutaric acidemia, oxidative stress, lipid peroxidation and striatal degeneration observed in association with severe dystonic-dyskinetic movement disorder. "
2.	Neuroinflammatory Diseases 01/01/2021 - "Protective effects of L-carnitine on behavioral alterations and neuroinflammation in striatum of glutaryl-COA dehydrogenase deficient mice."
3.	Glutaric Acidemia I 08/24/2023 - "Glutaric acidemia type 1 (GA1) is a neurotoxic metabolic disorder due to glutaryl-CoA dehydrogenase (GCDH) deficiency. " 08/24/2023 - "Glutaryl-CoA Dehydrogenase Misfolding in Glutaric Acidemia Type 1." 05/01/2022 - "Glutaric acidemia type 1 (GA1) is a treatable neurometabolic disorder caused by biallelic variants in the glutaryl-CoA dehydrogenase (GCDH) gene. " 09/01/2021 - "A novel mutation in the glutaryl-CoA dehydrogenase gene (GCDH) in an Iranian patient affected with Glutaric acidemia type 1." 01/01/2019 - "Glutaric acidemia type 1 (GA1) is an inherited metabolic autosomal recessive disorder that is caused by a deficiency in glutaryl-CoA dehydrogenase (GCDH). "
4.	Metabolic Diseases (Metabolic Disease) 01/01/2017 - "Glutaricaciduria type 1 (GA1), is a rare, treatable neuro metabolic disease, due to glutaryl- CoA dehydrogenase (GCDH) gene mutation.In" 12/22/2015 - "Glutaric aciduria type I (GA-I) is an inherited metabolic disease due to deficiency of glutaryl-CoA dehydrogenase (GCDH). " 03/01/2011 - "Glutaric acidemia type 1 (GA1) is a metabolic disease caused by a deficiency of glutaryl-CoA dehydrogenase (GCDH). " 10/01/2017 - "Glutaric acidemia I (GAI) is an autosomal recessive metabolic disease caused by a deficiency of glutaryl-CoA dehydrogenase enzyme (GCDH). " 01/01/2017 - "Glutaric aciduria type I (GA-I; synonym, glutaric acidemia type I) is a rare inherited metabolic disease caused by deficiency of glutaryl-CoA dehydrogenase located in the catabolic pathways of L-lysine, L-hydroxylysine, and L-tryptophan. "
5.	Dystonia (Limb Dystonia) 03/01/2012 - "Exome sequencing identifies GCDH (glutaryl-CoA dehydrogenase) mutations as a cause of a progressive form of early-onset generalized dystonia." 07/01/2004 - "Glutaryl-CoA dehydrogenase (GCDH) deficiency causes glutaric academia type I (GA-I), an inborn error of metabolism that is characterized clinically by dystonia and dyskinesia and pathologically by neural degeneration of the caudate nucleus and putamen. " 09/01/2000 - "Glutaryl-CoA dehydrogenase (GCDH) deficiency causes glutaric aciduria type I (GA I), an inborn error of metabolism that is characterized clinically by dystonia and dyskinesia and pathologically by neural degeneration of the caudate and putamen. " 01/01/1987 - "Glutaric acidemia, which is due to inherited deficiency of glutaryl-CoA dehydrogenase, is characterized clinically by progressive dystonia and dyskinesia in childhood, and pathologically by degeneration of the caudate and putamen. " 02/01/2000 - "Glutaryl-CoA dehydrogenase (GCDH) deficiency causes glutaric aciduria type I (GA I), an inborn error of metabolism that is characterized clinically by dystonia and dyskinesia, biochemically by excretion of glutaric and 3-hydroxyglutaric acids in urine, and pathologically by neural degeneration of the caudate and putamen. "

Related Drugs and Biologics

1.	Enzymes
2.	Proteins (Proteins, Gene)
3.	RNA (Ribonucleic Acid)
4.	Thioredoxin Reductase 1
5.	Carnitine (L-Carnitine)
6.	Antioxidants
7.	biliverdin reductase
8.	arsenite
9.	Lysine (L-Lysine)
10.	glutaric acid

Related Therapies and Procedures

1.	Secondary Prevention
2.	Role Playing (Playing, Role)