BCKDK Antibody
| Code | Size | Price |
|---|
| PSI-62-652-400ul | 400ul | £626.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: Rabbit Ig
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Human
- Mouse
Application: Western Blot (WB)
Storage:
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Documents
Further Information
Additional Names:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
Application Note:
For WB starting dilution is: 1:1000
Background:
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 ubiquitiously 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.
Buffer:
Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration:
batch dependent
Conjugate:
Unconjugated
DISCLAIMER:
Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Homology:
Predicted species reactivity based on immunogen sequence: Rat
Immunogen:
This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 325-356 amino acids from the C-terminal region of human BCKDK.
NCBI Gene ID #:
10295
NCBI Official Name:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
NCBI Official Symbol:
BCKDK
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
46 kDa
Protein Accession #:
O14874
Purification:
This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis
Research Area:
Obesity,Signal Transduction
Swissprot #:
O14874
User NOte:
Optimal dilutions for each application to be determined by the researcher.