Recommended positive controls: 293T, mouse brain, rat brain.
Predicted reactivity: Mouse (99%), Rat (99%), Zebrafish (85%), Xenopus laevis (89%), Rhesus Monkey (98%), Bovine (98%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKD-kinase; BCKDHKIN; branched chain alpha-ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; mitochondrial
Gene Aliases: AI327402; BCKDK; BCKDKD; BDK