Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Kits ELISA

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. De plus, nous expédions DBT Anticorps (30) et DBT Protéines (4) et beaucoup plus de produits pour cette protéine.

list all ELISA KIts Gène GeneID UniProt
DBT 1629 P11182
DBT 13171 P53395
Anti-Rat DBT DBT 29611  
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Top DBT Kits ELISA sur anticorps-enligne.fr

Showing 3 out of 8 products:

Catalogue No. Reactivité Sensibilité Gamme Images Quantité Livraison Prix Détails
Humain 7.8 pg/mL 31.25-2000 pg/mL Typical standard curve 96 Tests 15 to 18 Days
$910.56
Détails
Souris < 0.14 ng/mL 0.312 ng/mL - 20 ng/mL   96 Tests 11 to 18 Days
$731.99
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Boeuf (Vache)
  96 Tests 15 to 18 Days
$1,029.60
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Plus Kits ELISA pour DBT partenaires d'interaction

Human Dihydrolipoamide Branched Chain Transacylase E2 (DBT) interaction partners

  1. The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved.

  2. Deletion in DBT gene is associated with maple syrup urine disease.

  3. 4 novel mutations in DBT gene resulting in intermittent maple syrup urine disease in 7 Norwegian patients; pathogenic effect of the mutations is depletion of cellular protein; intermittent form of MSUD appears to be due to residual R301C mutant protein

  4. a distinct subset of antimitochondrial antibodies recognize sequences on branched-chain acyltransferase which located outside of the lipoyl binding domain, in primary biliary cirrhosis and overlap syndrome with autoimmune hepatitis

  5. presence of the interdomain linker restricts the motional freedom of the hbSBD more significantly than hbLBD, and that the linker region likely exists as a soft rod rather than a flexible string in solution.

  6. in our cohort more severe enzyme & clinical phenotypes of variant maple syrup urine disease were mainly associated with specific genotypes in BCKDHA gene; milder enzyme & clinical phenotypes were associated with specific genotypes in BCKDHB & DBT genes

  7. 30 Maple syrup urine disease Portuguese patients studied; 17 putative mutations have been identified (6 in BCKDHA, 5 in BCKDHB and 6 in DBT); 7 of are described for the first time.

  8. Examination of the deletion mutation in the E2 (DBT) gene facilitated early MSUD diagnosis and was beneficial for the determination of the proper course of treatment.

  9. In 37% (12 patients) of a total of 64 alleles, the supposed maple syrup urine disease-causing mutations in Turkish patients were located in the BCKDHA gene, in 44% (14 patients) in the BCKDHB gene and in 19% (6 patients) in the DBT gene.

  10. two novel type IB MSUD mutations in Israeli patients, which affect the E1beta subunit in the decarboxylase (E1) component of the branched-chain alpha-ketoacid dehydrogenase complex

  11. Mutation in DBT causes a subset of maple syrup urine disease in Ashkenazi Jewish population.

Zebrafish Dihydrolipoamide Branched Chain Transacylase E2 (DBT) interaction partners

  1. que mutant larvae exhibit a progressive locomotor defect that culminates in unusual nose-to-tail compressions and an inability to swim. Peripheral nerve recordings demonstrate abnormal locomotor output to the axial muscles used for swimming.

Cow (Bovine) Dihydrolipoamide Branched Chain Transacylase E2 (DBT) interaction partners

  1. This gating mechanism synchronizes the binding of the two substrates to the active-site channel, which serves as a feed-forward switch to coordinate the E2b-catalyzed acyltransfer reaction.

DBT profil antigène

Antigen Summary

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.

Gene names and symbols associated with DBT

  • dihydrolipoamide branched chain transacylase E2 (DBT) anticorps
  • dihydrolipoamide branched chain transacylase E2 (dbt) anticorps
  • dihydrolipoamide branched chain transacylase E2 (CpipJ_CPIJ006326) anticorps
  • dihydrolipoamide branched chain transacylase E2 (BDBG_05874) anticorps
  • dihydrolipoamide branched chain transacylase E2 (NAEGRDRAFT_78509) anticorps
  • dihydrolipoamide branched chain transacylase E2 (VDBG_04820) anticorps
  • hypothetical protein (PGTG_17722) anticorps
  • dihydrolipoamide branched chain transacylase E2 (Dbt) anticorps
  • dihydrolipoamide branched chain transacylase E2 L homeolog (dbt.L) anticorps
  • BCATE2 anticorps
  • BCKAD-E2 anticorps
  • BCKADE2 anticorps
  • D3Wsu60e anticorps
  • E2 anticorps
  • E2b anticorps
  • im:7147214 anticorps
  • zgc:103768 anticorps

Protein level used designations for DBT

lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial , dihydrolipoamide branched chain transacylase E2 , BCKAD E2 subunit , E2 component of branched chain alpha-keto acid dehydrogenase complex , branched chain acyltransferase, E2 component , branched-chain alpha-keto acid dehydrogenase complex component E2 , dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex , dihydrolipoyl transacylase , dihydrolipoyllysine-residue (2-methylpropanoyl)transferase , lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex , mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b) , BCKAD E2 , BCKAD-E2 , BCKADE2 , branched-chain alpha-ketoacid dehydrogenase, E2 subunit , part of the BCKAD complex , que , mitochondrial branched-chain alpha-keto acid dihydrolipoyl acyltransferase , component of branched chain keto acid dehydrogenase complex

GENE ID SPECIES
457056 Pan troglodytes
100174565 Pongo abelii
549717 Xenopus (Silurana) tropicalis
711547 Macaca mulatta
100050317 Equus caballus
100156530 Sus scrofa
100346987 Oryctolagus cuniculus
6038202 Culex quinquefasciatus
8503735 Ajellomyces dermatitidis SLH14081
8862102 Naegleria gruberi strain NEG-M
9530433 Verticillium alfalfae VaMs.102
10529473 Puccinia graminis f. sp. tritici CRL 75-36-700-3
1629 Homo sapiens
13171 Mus musculus
29611 Rattus norvegicus
541388 Danio rerio
447616 Xenopus laevis
395374 Gallus gallus
479929 Canis lupus familiaris
280759 Bos taurus
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