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 (73) und und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
GCDH 2639 Q92947
Ratte GCDH GCDH 364975  
GCDH 270076 Q60759
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Top Glutaryl-CoA Dehydrogenase Proteine auf antikoerper-online.de

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
$6,869.04
Details
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
$6,869.04
Details
Escherichia coli (E. coli) Human His tag   50 μg Anmelden zum Anzeigen 4 Days
$385.00
Details
Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 11 bis 12 Tage
$414.29
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HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Anmelden zum Anzeigen Verfügbar
$814.00
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Escherichia coli (E. coli) Human Unkonjugiert SDS-PAGE analysis of Human GCDH Protein. 100 μg Anmelden zum Anzeigen 11 bis 18 Tage
$511.69
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Escherichia coli (E. coli) Maus Unkonjugiert SDS-PAGE analysis of Mouse GCDH Protein. 100 μg Anmelden zum Anzeigen 11 bis 18 Tage
$540.11
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Escherichia coli (E. coli) Human His tag 50 μg Anmelden zum Anzeigen 15 bis 19 Tage
$492.31
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Hefe Rind (Kuh) His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
$3,089.17
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Hefe Cynomolgus His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
$3,089.17
Details

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

  7. 29 GCDH mutations were identified in 23 glutaric aciduria type 1 patients, including 11 novel mutations

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

  9. These cells displayed decreased levels of GCDH tetramer.

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

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

  1. 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.

  2. Using an experimental model with a phenotype similar to that of GA-I in humans-the Gcdh(-/-) mice under high lysine diet (Gcdh(-/-) -Lys (zeige LYZ Proteine))-we provide evidence that a reduction in cortical inhibition of Gcdh(-/-) -Lys (zeige LYZ Proteine) mice, probably induced by GAD (zeige GAD1 Proteine) dysfunction, leads to hyperexcitability and increased slow oscillations associated with neurologic abnormalities in glutaric academia type I.

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

  4. GCDH-defective astrocytes actively contribute to produce and accumulate GA and 3HGA when Lys (zeige LYZ Proteine) catabolism is stressed

  5. 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.

  6. 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.

  7. These results provide evidence that glutamate (zeige GRIN1 Proteine) receptor and transporter expression is higher in Gcdh-/- mice.

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

  9. 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

GENE ID SPEZIES
2639 Homo sapiens
476696 Canis lupus familiaris
364975 Rattus norvegicus
506310 Bos taurus
393860 Danio rerio
270076 Mus musculus
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