glu·ta·ryl-CoA de·hy·dro·gen·ase

an enzyme that catalyzes the reaction of glutaryl-CoA with an acceptor to form crotonoyl-CoA, CO₂, and the reduced acceptor; a deficiency of this enzyme will lead to either glutaric acidemia type I or hyperoxaluria type II.

Farlex Partner Medical Dictionary © Farlex 2012

GCDH

A gene on chromosome 19p13.2 that encodes glutaryl-CoA dehydrogenase, a member of the acyl-CoA dehydrogenase family, which catalyses the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2 in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.

Molecular pathology
Defects in GCDH cause glutaric aciduria type 1.

Mentioned in

GCDH

References in periodicals archive

Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I).

Late-onset neurologic disease in glutaryl-CoA dehydrogenase deficiency.

Glutaric Aciduria Type I Diagnosis Case with Normal Glutaryl Carnitine and Urine Organic Acid Analysis

1) is notable for lactic and glutaric acid peaks, secondary to diminished perfusion and defective glutaryl-CoA dehydrogenase activities, respectively.

A Term Newborn with Respiratory Distress, Acidosis, and Hypoglycemia

Glutaric aciduria type 1 (GA-1) is an autosomal recessive disorder caused by deficiency of glutaryl-CoA dehydrogenase, which is the key mitochondrial enzyme involved in the final degradation of lysine, L-hydroxylysine and L-tryphtophan.

Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency.

Teaching NeuroImages: Glutaric aciduria type 1 (glutaryl-CoA dehydrogenase deficiency).

Glutaric aciduria type 1 - importance of early diagnosis and treatment

Glutaric aciduria type 1 is the most common OA in South Africa and is caused by AR deficiency of glutaryl-CoA dehydrogenase (GCDH), a critical enzyme in the infantile neuronal lysine catabolic pathway.

(1.) van der Watt G, Owen EP, Berman P, et al.Glutaric aciduria type 1 in South Africa-high incidence of glutaryl-CoA dehydrogenase deficiency in black South Africans.

Investigation of metabolic encephalopathy: encephalopathy may be a presenting sign in a wide range of medical conditions

Assignment of human glutaryl-CoA dehydrogenase gene (GCDH) to the short arm of chromosome 19 (19p13.2) by in situ hybridization and somatic cell hybrid analysis.

Bilateral temporal arachnoid cysts and glutaric aciduria type 1/iki tarafli temporal araknoid kistler ve glutarik asiduri tip 1

Screening the urine for a deficiency of glutaryl-CoA dehydrogenase makes the diagnosis.

Certain injuries highly specific to child abuse. (Don't Jump to Conclusions)