Promotional price valid on web orders only. Your contract pricing may differ. Interested in signing up for a dedicated account number?
Learn More
  1. Home
  2. Products
  3. Antibodies
  4. Primary Antibodies
  5. All Primary Antibodies
  6. PIMA544776

Invitrogen™ BCKDK Recombinant Rabbit Monoclonal Antibody (JE63-77)
GREENER_CHOICE

Catalog No. PIMA544776
Change view
Click to view available options
Quantity:
100 μL
Catalog No. Quantity
PIMA544776 100 μL
1 options
This item is not returnable. View return policy

Catalog No. PIMA544776

Supplier: Invitrogen™ MA544776

Only null left
Add to Cart
Add to Cart
This item is not returnable. View return policy

Description

Rabbit Recombinant Monoclonal Antibody

BCKDK Recombinant Monoclonal Antibody for Western Blot

The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
TRUSTED_SUSTAINABILITY

Specifications

Antigen BCKDK
Applications Western Blot
Classification Recombinant Monoclonal
Clone JE63-77
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation TBS with 0.05% BSA, 40% glycerol and 0.05% sodium azide; pH 7.4
Gene BCKDK
Gene Accession No. O14874
Gene Alias [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; AI327402; BCKDHKIN; BCKDK; BCKDKD; BCKD-kinase; BDK; branched chain alpha-ketoacid dehydrogenase kinase; branched chain ketoacid dehydrogenase kinase; branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
Gene Symbols BCKDK
Host Species Rabbit
Immunogen Synthetic peptide within human BCKDK aa 50-100/412.
Purification Method Protein A
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 10295
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
Show More Show Less

Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov
Product Title
Select an issue

By clicking Submit, you acknowledge that you may be contacted by Fisher Scientific in regards to the feedback you have provided in this form. We will not share your information for any other purposes. All contact information provided shall also be maintained in accordance with our Privacy Policy.