Further Information
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
For WB starting dilution is: 1:1000
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.
Supplied in PBS with 0.09% (W/V) sodium azide.
batch dependent
Unconjugated
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.
Predicted species reactivity based on immunogen sequence: Rat
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.
10295
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
BCKDK
Homo sapiens
Liquid
PREDICTED MOLECULAR WEIGHT:
46 kDa
O14874
This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis
Obesity,Signal Transduction
O14874
Optimal dilutions for each application to be determined by the researcher.