Gene Symbol: Gcdh
Description: glutaryl-CoA dehydrogenase
Alias: glutaryl-CoA dehydrogenase, mitochondrial, glutaryl-Coenzyme A dehydrogenase
Keyser B, Mühlhausen C, Dickmanns A, Christensen E, Muschol N, Ullrich K, et al
. Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH). Hum Mol Genet. 2008;17:3854-63 pubmed publisher
..GA1) is an autosomal recessive neurometabolic disorder caused by mutations in the glutaryl-CoA dehydrogenase gene (GCDH), leading to an accumulation and high excretion of glutaric acid and 3-hydroxyglutaric acid...
Reichmann H, Maltese W, DeVivo D. Enzymes of fatty acid beta-oxidation in developing brain. J Neurochem. 1988;51:339-44 pubmed
Gao J, Zhang C, Fu X, Yi Q, Tian F, Ning Q, et al
. Effects of targeted suppression of glutaryl-CoA dehydrogenase by lentivirus-mediated shRNA and excessive intake of lysine on apoptosis in rat striatal neurons. PLoS ONE. 2013;8:e63084 pubmed publisher
In glutaric aciduria type 1 (GA1), glutaryl-CoA dehydrogenase (GCDH) deficiency has been shown to be responsible for the accumulation of glutaric acid and striatal degeneration...
Biagosch C, Ediga R, Hensler S, Faerberboeck M, Kuehn R, Wurst W, et al
. Elevated glutaric acid levels in Dhtkd1-/Gcdh- double knockout mice challenge our current understanding of lysine metabolism. Biochim Biophys Acta Mol Basis Dis. 2017;1863:2220-2228 pubmed publisher
..is a rare organic aciduria caused by the autosomal recessive inherited deficiency of glutaryl-CoA dehydrogenase (GCDH)...
Goodman S. Organic aciduria in the riboflavin-deficient rat. Am J Clin Nutr. 1981;34:2434-7 pubmed
MaÅ‚ecki J, Ho A, Moen A, Dahl H, Falnes P. Human METTL20 is a mitochondrial lysine methyltransferase that targets the Î² subunit of electron transfer flavoprotein (ETFÎ²) and modulates its activity. J Biol Chem. 2015;290:423-34 pubmed publisher
..Based on the previous naming of similar enzymes, we suggest the renaming of human METTL20 to ETFÎ²-KMT. ..
Braissant O, Jafari P, Remacle N, Cudré Cung H, Do Vale Pereira S, Ballhausen D. Immunolocalization of glutaryl-CoA dehydrogenase (GCDH) in adult and embryonic rat brain and peripheral tissues. Neuroscience. 2017;343:355-363 pubmed publisher
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial enzyme that is involved in the degradation of tryptophan, lysine and hydroxylysine. Deficient enzyme activity leads to glutaric aciduria type-I (GA-I)...