Glutaryl-CoA Dehydrogenase 蛋白 (GCDH)

The protein encoded by GCDH belongs to the acyl-CoA dehydrogenase family. 再加上,我们可以发Glutaryl-CoA Dehydrogenase 抗体 (73)Glutaryl-CoA Dehydrogenase 试剂盒 (4)和数多这个蛋白质的别的产品。

列出全部蛋白 基因 基因ID UniProt
GCDH 2639 Q92947
大鼠 GCDH GCDH 364975  
GCDH 270076 Q60759
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Insect Cells His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
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GCDH 蛋白 by Origin and Source

Origin 在表达 标记
Human , , ,
, , ,
Mouse (Murine) ,
,

更多Glutaryl-CoA Dehydrogenase (GCDH)互动伙伴

Human Glutaryl-CoA Dehydrogenase (GCDH) interaction partners

  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) interaction partners

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

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

  3. 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.

  4. 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.

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

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

  7. 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.

  8. 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.

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

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

  11. 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.

蛋白简介Glutaryl-CoA Dehydrogenase (GCDH)

蛋白简介

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.

Gene names and symbols associated with GCDH

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

Protein level used designations for GCDH

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

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