Sign in or Register   Sign in or Register
  |  

Mouse Anti-DBT Recombinant Antibody (1G2) (CBMAB-D0315-YC)

Provided herein is a Mouse monoclonal antibody, which binds to Dihydrolipoamide Branched Chain Transacylase E2 (DBT). The antibody can be used for immunoassay techniques, such as WB, FC.
See all DBT antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1G2
Antibody Isotype
IgG1
Application
WB, FC

Basic Information

Immunogen
Full length human recombinant protein of human DBT(NP_001909) produced in HEK293T cell
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Dihydrolipoamide Branched Chain Transacylase E2
Introduction
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.
Entrez Gene ID
1629
UniProt ID
P11182
Alternative Names
Dihydrolipoamide Branched Chain Transacylase E2; Dihydrolipoyllysine-Residue (2-Methylpropanoyl)Transferase; Lipoamide Acyltransferase Component Of Branched-Chain Alpha-Keto Acid Dehydrogenase Complex, Mitochondrial; Dihydrolipoamide Acetyltransferase Component Of Branched-Chain Alpha-Keto Acid Dehydrogenase Complex; Branched-Chain Alpha-Keto Acid Dehydrogenase Complex Component E2; 52 KDa Mitochondrial Autoantigen Of Primary Biliary Cirrhosis; Branched Chain 2-Oxo-Acid Dehydrogenase Complex Component E2; BCOADC-E2; BCKAD-E2; BCKADE2; BCATE2; Dihydrolipoamide Branched Chain Transacylase (E2 Component Of Branched Chain Keto Acid Dehydrogenase Complex; Maple Syrup Urine Disease);
Function
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
Biological Process
Branched-chain amino acid catabolic process Source: Reactome
Cellular Location
Mitochondrion matrix
Involvement in disease
Patients with primary biliary cirrhosis (PBC) show autoantibodies against the E2 component of branched-chain alpha-keto acid dehydrogenase complex. PBC is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis.
Maple syrup urine disease 2 (MSUD2):
A metabolic disorder due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration. Clinical features include mental and physical retardation, feeding problems, and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine. If untreated, maple syrup urine disease can lead to seizures, coma, and death. The disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still involve developmental delay and other medical problems if not treated.
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-DBT Recombinant Antibody (1G2)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare