BCKDK Polyclonal Antibody, Invitrogen™
Manufacturer: Thermo Scientific
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | PIPA553697-Each-of-1 | In Stock | ₹ 48,905.50 |
PIPA553697 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 48,905.50
GST (18%): ₹ 8,802.99
Total Price: ₹ 57,708.49
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
Antigen affinity chromatography
Regulatory Status
RUO
Gene ID (Entrez)
10295, 12041, 29603
Content And Storage
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Form
Liquid
Applications
Immunohistochemistry, Western Blot
Concentration
0.3 mg/mL
Formulation
PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Gene Accession No.
O14874, O55028, Q00972
Gene Symbols
BCKDK
Immunogen
Recombinant protein corresponding to Human BCKDK
Quantity
100 μL
Primary or Secondary
Primary
Target Species
Human, Mouse, Rat
Product Type
Antibody
Isotype
IgG
Related Products
Description
- Immunogen sequence: LLDDHKDVVT LLAEGLRESR KHIEDEKLVR YFLDKTLTSR LGIRMLATHH LALHEDKPDF VGIICTRLSP KKIIEKWVDF ARRLCEHKYG NAPRVRINGH VAARFPFIPM PLDY Highest antigen sequence identity to the following orthologs: Mouse - 99%, Rat - 100%
- 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.