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D2-Hydroxyglutarate Dehydrogenase (D2HGDH)

D2HGD

D2HGDH encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features.
Subcellular fractionation of rat livers indicated that rat D2hgdh was present in the mitochondrial fraction. Assays of D-2-hydroxyglutarate dehydrogenase in rat tissues indicated that this enzyme is most active in liver and kidney but is also active in heart and brain .

Organism species: Homo sapiens (Human)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Hu01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Hu01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Hu01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Hu01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Hu01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Mu01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Mu01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Mu01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Mu01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Mu01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Ra01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Ra01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Ra01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Ra01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Ra01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Ca01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Ca01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Ca01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Ca01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Ca01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Po01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Po01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Po01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Po01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Po01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Bo01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Bo01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Bo01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Bo01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Bo01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Rb01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Rb01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Rb01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Rb01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Rb01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO447Mo01 Recombinant D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO447Mo01 Monoclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IP.
PAO447Mo01 Polyclonal Antibody to D2-Hydroxyglutarate Dehydrogenase (D2HGDH) WB; IHC; ICC; IF.
Assay Kits CLO447Mo01 CLIA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) CLIA assay for Antigen Detection
ELO447Mo01 ELISA Kit for D2-Hydroxyglutarate Dehydrogenase (D2HGDH) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

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

"Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
Nature 434:724-731(2005) [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]

"Identification of a dehydrogenase acting on D-2-hydroxyglutarate."
Biochem. J. 381:35-42(2004) [PubMed] [Europe PMC] [Abstract]

"Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause D-2-hydroxyglutaric aciduria."
Am. J. Hum. Genet. 76:358-360(2005) [PubMed] [Europe PMC] [Abstract]

"Mutations in phenotypically mild D-2-hydroxyglutaric aciduria."
Ann. Neurol. 58:626-630(2005) [PubMed] [Europe PMC] [Abstract]

"Phenotypic heterogeneity in the presentation of D-2-hydroxyglutaric aciduria in monozygotic twins."
Mol. Genet. Metab. 86:200-205(2005) [PubMed] [Europe PMC] [Abstract]

"D-2-Hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?"
Mol. Genet. Metab. 88:53-57(2006) [PubMed] [Europe PMC] [Abstract]