Glutaryl-CoA Dehydrogenase Proteine (GCDH)

The protein encoded by GCDH belongs to the acyl-CoA dehydrogenase family. Zusätzlich bieten wir Ihnen Glutaryl-CoA Dehydrogenase Antikörper (75) und Glutaryl-CoA Dehydrogenase Kits (4) und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
GCDH 2639 Q92947
Ratte GCDH GCDH 364975  
GCDH 270076 Q60759
Direkt bei antikoerper-online bestellen
  • +1 877 302 8632
  • +1 888 205 9894 (toll-free)
  • Online bestellen

Top Glutaryl-CoA Dehydrogenase Proteine auf

Showing 10 out of 17 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
Escherichia coli (E. coli) Human His tag   50 μg Anmelden zum Anzeigen 4 Days
Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 11 bis 12 Tage
HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Anmelden zum Anzeigen 11 Days
Escherichia coli (E. coli) Human Unkonjugiert SDS-PAGE analysis of Human GCDH Protein. 100 μg Anmelden zum Anzeigen 11 bis 18 Tage
Escherichia coli (E. coli) Maus Unkonjugiert SDS-PAGE analysis of Mouse GCDH Protein. 100 μg Anmelden zum Anzeigen 11 bis 18 Tage
Escherichia coli (E. coli) Human His tag 50 μg Anmelden zum Anzeigen 15 bis 19 Tage
Hefe Cynomolgus His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
Hefe Rind (Kuh) His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage

GCDH Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Human , , ,
, , ,
Mouse (Murine) ,

Weitere Proteine zu Glutaryl-CoA Dehydrogenase (GCDH) Interaktionspartnern

Human Glutaryl-CoA Dehydrogenase (GCDH) Interaktionspartner

  1. Molecular genetics analysis identified 14 different mutations in the GCDH gene in the 18 patients with Glutaric acidemia I

  2. Four mutations of the glutaryl-CoA dehydrogenase (GCDH) gene were identified among the patients with diagnosis of glutaric acidemia type I (GA-I).

  3. We report the allele frequencies for three known Glutaric aciduria type I low excretors GCDH variants (M405V, V400M and R227P) and note that both the M405V and V400M variants are significantly more common in the population of African ancestry compared to the general population

  4. Our data underscore the impact of GCDH protein interactions mediated by amino acid residues on the surface of GCDH required for proper enzymatic activity

  5. Mutations in GCDH gene observed in the present study indicate genetic heterogeneity in GCDH gene among South Indian population. No definite genotype-phenotype correlations were observed.

  6. 2 novel mutations, p.Glu64Asp and p.Gly268Val, account for majority of disease alleles in Cypriot patients with Glutaric aciduria type I; a founder effect for the p.Glu64Asp and the p.Gly268Val can be suggested based on place of origin of mutation carriers

  7. Point mutation of GCDH gene is associated with glutaric academia type I.

  8. 29 GCDH mutations were identified in 23 glutaric aciduria type 1 patients, including 11 novel mutations

  9. Data indicate a homozygous c.1244-2A> C mutation of the glutaryl-CoA dehydrogenase (GCDH) gene in both patients.

  10. These cells displayed decreased levels of GCDH tetramer.

  11. Identification of GCDH gene mutations in four patients with glutaric academia type I.

  12. A homozygous, disease-segregating mutation (p.Val400Met) was identified in the glutaryl-CoA dehydrogenase (GCDH) gene at chromosome 19p13.

  13. physiological concentrations of flavin adenine dinucleotide resulted in a spectacular enhancement of the thermal stabilities of GCDH and prevented enzymatic activity loss

  14. GCDH gene mutations are identified in 8 patients with glutaric aciduria type I

  15. 12 glutaric aciduria type 1 patients were found homozygous for the same A293T mutation in the glutaryl-CoA dehydrogenase (GCDH) gene.

  16. mutational analysis of glutaryl-CoA dehydrogenase in two patients with glutaric aciduria type 1.

  17. Cerebral toxicity caused by GCDH deficiency may induce a state of arteriolar dilation and increased cerebral blood volume.

  18. Three-dimensional structures of human GCD in uncomplexed form and in complex with 4-nitrobutyryl-CoA are reported, and the structural bases for the mechanisms of the dehydrogenation and decarboxylation reactions are proposed.

  19. The authors report two GCDH-deficient patients with macrocephaly presenting with progressive neurologic deterioration and a severe leukoencephalopathy during adolescence or adulthood.

  20. An autosomal recessive disease thsat leads to an accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitine deficiency, (review)

Mouse (Murine) Glutaryl-CoA Dehydrogenase (GCDH) Interaktionspartner

  1. myelin damage seen in Gcdh-/- mice fed with 2.8% Lys seems to be mediated by a long-term increased levels of Glutaric acidemia type I metabolites having deleterious effects in myelinating oligodendrocytes over neurons.

  2. The present data indicate that QA significantly contributes to the histopathological changes observed in the striatum of Gcdh-/- mice.

  3. Elevated glutaric acid levels in Dhtkd1-/Gcdh- double knockout mice challenge our current understanding of lysine metabolism.

  4. Quinolinic acid increased lactate release in both Gcdh (+/+) and Gcdh (-/-) mice and reduced the activities of complex IV and creatine kinase only in the striatum of Gcdh (-/-) mice.

  5. Using an experimental model with a phenotype similar to that of GA-I in humans-the Gcdh(-/-) mice under high lysine diet (Gcdh(-/-) -Lys)-we provide evidence that a reduction in cortical inhibition of Gcdh(-/-) -Lys mice, probably induced by GAD dysfunction, leads to hyperexcitability and increased slow oscillations associated with neurologic abnormalities in glutaric academia type I.

  6. The data of this study indicate higher susceptibility of Gcdh(-/-) mice to excitotoxic damage.

  7. GCDH-defective astrocytes actively contribute to produce and accumulate GA and 3HGA when Lys catabolism is stressed

  8. L-pipecolate is a major degradation product from L-lysine in murine brain generated by alpha-deamination of this amino acid in Gcdh-deficient mouse model for glutaric aciduria type I.

  9. Data show that glutaryl-Coenzyme A dehydrogenase Gcdh(-/-) knockout mice display elevated levels of glutaric acid (GA) and 3-hydroxyglutaric acid (3-OHGA) but do not spontaneously develop striatal lesions.

  10. These results provide evidence that glutamate receptor and transporter expression is higher in Gcdh-/- mice.

  11. bioenergetic impairment may play an important role in the pathomechanisms underlying neurodegenerative changes in glutaryl-CoA dehydrogenase deficiency

  12. Data show that pathologic events in glutaryl-CoA dehydrogenase-deficient mice/glutaric acidemia type I begin in neurons while lysine accumulation in the immature brain allows increased glutaric acid production and age-dependent injury.

Glutaryl-CoA Dehydrogenase (GCDH) Protein Überblick

Protein Überblick

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. 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. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12.

Genbezeichner und Symbole assoziert mit GCDH

  • glutaryl-CoA dehydrogenase (GCDH)
  • glutaryl-CoA dehydrogenase (Gcdh)
  • glutaryl-CoA dehydrogenase a (gcdha)
  • glutaryl-Coenzyme A dehydrogenase (Gcdh)
  • 9030411L18 Protein
  • ACAD5 Protein
  • AI266902 Protein
  • D17825 Protein
  • GCD Protein
  • zgc:56505 Protein
  • zgc:77704 Protein

Bezeichner auf Proteinebene für GCDH

glutaryl-CoA dehydrogenase, mitochondrial , glutaryl-Coenzyme A dehydrogenase , GCD

2639 Homo sapiens
476696 Canis lupus familiaris
364975 Rattus norvegicus
506310 Bos taurus
393860 Danio rerio
270076 Mus musculus
Ausgewählte Anbieter für Glutaryl-CoA Dehydrogenase Proteine (GCDH)
Haben Sie etwas anderes gesucht?