BCKDK Polyclonal Antibody, Invitrogen™
Manufacturer: Thermo Scientific
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | PIPA5104476-Each-of-1 | In Stock | ₹ 46,502.50 |
PIPA5104476 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 46,502.50
GST (18%): ₹ 8,370.45
Total Price: ₹ 54,872.95
Antigen
BCKDK
Classification
Polyclonal
Conjugate
Unconjugated
Gene
BCKDK
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
Host Species
Rabbit
Purification Method
Affinity chromatography
Regulatory Status
RUO
Gene ID (Entrez)
10295, 12041, 29603
Content And Storage
-20°C
Form
Liquid
Applications
Immunocytochemistry, Western Blot
Concentration
1 mg/mL
Formulation
PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
Gene Accession No.
O14874, O55028, Q00972
Gene Symbols
BCKDK
Immunogen
A synthesized peptide derived from human BCKDK(Accession O14874), corresponding to amino acid residues L21-T71.
Quantity
100 μL
Primary or Secondary
Primary
Target Species
Human, Mouse, Rat
Product Type
Antibody
Isotype
IgG
Related Products
Description
- Antibody detects endogenous levels of total BCKD
- 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.