You are viewing an incomplete version of our website. Please click to reload the website as full version.

Glutaryl-CoA Dehydrogenase Proteins (GCDH)

The protein encoded by GCDH belongs to the acyl-CoA dehydrogenase family. Additionally we are shipping Glutaryl-CoA Dehydrogenase Antibodies (53) and Glutaryl-CoA Dehydrogenase Kits (1) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
GCDH 2639 Q92947
Rat GCDH GCDH 364975  
GCDH 270076 Q60759
How to order from antibodies-online
  • +1 877 302 8632
  • +1 888 205 9894 (toll-free)
  • Order online
  • orders@antibodies-online.com

Top Glutaryl-CoA Dehydrogenase Proteins at antibodies-online.com

Showing 10 out of 15 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Human His tag 50 μg Log in to see 7 to 8 Days
$407.00
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$3,089.17
Details
Yeast Macaca fascicularis His tag   1 mg Log in to see 56 to 66 Days
$3,089.17
Details
Yeast Wild boar His tag   1 mg Log in to see 56 to 66 Days
$3,091.00
Details
HOST_Escherichia coli (E. coli) Human His tag   1 mg Log in to see 3 to 4 Days
$5,329.50
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated   50 μg Log in to see 3 to 4 Days
$467.86
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

GCDH Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Mouse (Murine)

Top referenced Glutaryl-CoA Dehydrogenase Proteins

  1. Human GCDH Protein expressed in Escherichia coli (E. coli) - ABIN667797 : Keyser, Mühlhausen, Dickmanns, Christensen, Muschol, Ullrich, Braulke: Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH). in Human molecular genetics 2008 (PubMed)
    Show all 2 references for ABIN667797

More Proteins for Glutaryl-CoA Dehydrogenase (GCDH) Interaction Partners

Human Glutaryl-CoA Dehydrogenase (GCDH) interaction partners

  1. 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.

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

  3. 29 GCDH mutations were identified in 23 glutaric aciduria type 1 patients, including 11 novel mutations

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

  5. These cells displayed decreased levels of GCDH tetramer.

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

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

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

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

  10. 12 glutaric aciduria type 1 patients were found homozygous for the same A293T mutation in the glutaryl-CoA dehydrogenase (GCDH) gene.

Mouse (Murine) Glutaryl-CoA Dehydrogenase (GCDH) interaction partners

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

  2. GCDH-defective astrocytes actively contribute to produce and accumulate GA and 3HGA when Lys (show LYZ Proteins) catabolism is stressed

  3. 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.

  4. 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.

  5. These results provide evidence that glutamate (show GRIN1 Proteins) receptor and transporter expression is higher in Gcdh-/- mice.

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

  7. 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 Profile

Protein Summary

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-Coenzyme A dehydrogenase (gcdh)
  • glutaryl-Coenzyme A dehydrogenase (Gcdh)
  • 9030411L18 protein
  • ACAD5 protein
  • AI266902 protein
  • D17825 protein
  • GCD protein
  • zgc:56505 protein
  • zgc:77704 protein

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
Selected quality suppliers for Glutaryl-CoA Dehydrogenase Proteins (GCDH)
Did you look for something else?