Call Now

Glutaryl Coenzyme A Dehydrogenase (GCDH)

GCD; ACAD5; Glutaryl-CoA dehydrogenase, mitochondrial

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)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Hu01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Hu01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Hu01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Hu01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Hu01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Mu01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Mu01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Mu01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Mu01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Mu01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Ra01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Ra01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Ra01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Ra01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Ra01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Ca01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Ca01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Ca01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Ca01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Ca01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Po01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Po01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Po01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Po01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Po01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Bo01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Bo01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Bo01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Bo01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Bo01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Rb01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Rb01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Rb01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Rb01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Rb01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPT103Mo01 Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAT103Mo01 Monoclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IP.
PAT103Mo01 Polyclonal Antibody to Glutaryl Coenzyme A Dehydrogenase (GCDH) WB; IHC; ICC; IF.
Assay Kits CLT103Mo01 CLIA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) CLIA assay for Antigen Detection
ELT103Mo01 ELISA Kit for Glutaryl Coenzyme A Dehydrogenase (GCDH) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

"Pork and human cDNAs encoding glutaryl-CoA dehydrogenase."
Prog. Clin. Biol. Res. 375:169-173(1992) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biology of human chromosome 19."
Nature 428:529-535(2004) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"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]

"Kinetic mechanism of glutaryl-CoA dehydrogenase."
Biochemistry 45:15853-15861(2006) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"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]

"Glutaric aciduria type I in the Arab and Jewish communities in Israel."
Am. J. Hum. Genet. 59:1012-1018(1996) [PubMed] [Europe PMC] [Abstract]

"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]

"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]

"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]