Glutaryl Coenzyme A Dehydrogenase (GCDH)

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GCD; ACAD5; Glutaryl-CoA dehydrogenase, mitochondrial

Glutaryl Coenzyme A Dehydrogenase (GCDH)
Glutaryl-CoA dehydrogenase (GCDH) is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and tryptophan. Specifically, it is responsible for the dehydrogenation and decarboxylation of glutaryl-CoA to crotonyl-CoA in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. The active enzyme exists as a homotetramer in the mitochondrial matrix.It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Alternatively spliced transcript variants encoding different isoforms have been identified.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD681Hu01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAD681Hu01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA Kit Customized Service Offer
n/a ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD681Mu01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAD681Mu01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA Kit Customized Service Offer
n/a ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA Kit Customized Service Offer
n/a ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) ELISA Kit Customized Service Offer
  1. "Pork and human cDNAs encoding glutaryl-CoA dehydrogenase."Prog. Clin. Biol. Res. 375:169-173(1992) [PubMed] [Europe PMC] [Abstract]
  2. "Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli."Hum. Mol. Genet. 4:1493-1498(1995) [PubMed] [Europe PMC] [Abstract]
  3. "The human glutaryl-CoA dehydrogenase gene: report of intronic sequences and of 13 novel mutations causing glutaric aciduria type I."Hum. Genet. 102:452-458(1998) [PubMed] [Europe PMC] [Abstract]
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  5. "The DNA sequence and biology of human chromosome 19." Nature 428:529-535(2004) [PubMed] [Europe PMC] [Abstract]
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  7. "Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients."J. Clin. Invest. 73:778-784(1984) [PubMed] [Europe PMC] [Abstract]
  8. "Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations."Hum. Mutat. 12:141-144(1998) [PubMed] [Europe PMC] [Abstract]
  9. "Kinetic mechanism of glutaryl-CoA dehydrogenase."Biochemistry 45:15853-15861(2006) [PubMed] [Europe PMC] [Abstract]
  10. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  11. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  12. "Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions."Biochemistry 43:9674-9684(2004) [PubMed] [Europe PMC] [Abstract]
  13. "The effect of a Glu370Asp mutation in glutaryl-CoA dehydrogenase on proton transfer to the dienolate intermediate."Biochemistry 46:14468-14477(2007) [PubMed] [Europe PMC] [Abstract]
  14. "Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish."Am. J. Hum. Genet. 59:1006-1011(1996) [PubMed] [Europe PMC] [Abstract]
  15. "Glutaric aciduria type I in the Arab and Jewish communities in Israel."Am. J. Hum. Genet. 59:1012-1018(1996) [PubMed] [Europe PMC] [Abstract]
  16. "Severe phenotype despite high residual glutaryl-CoA dehydrogenase activity: a novel mutation in a Turkish patient with glutaric aciduria type I."J. Inherit. Metab. Dis. 26:713-714(2003) [PubMed] [Europe PMC] [Abstract]
  17. "Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH)."Hum. Mol. Genet. 17:3854-3863(2008) [PubMed] [Europe PMC] [Abstract]
  18. "Molecular analysis of Cypriot patients with Glutaric aciduria type I: Identification of two novel mutations."Clin. Biochem. 47:1300-1305(2014) [PubMed] [Europe PMC] [Abstract]