Thermo Fisher Scientific BCKDK Polyclonal Antibody

Applications

Western Blot (WB)

• Tested Dilution: 1:500–1:3,000

Immunohistochemistry (Paraffin) (IHC (P))

• Tested Dilution: 1:100–1:1,000

Product Specifications

Product Specific Information

• 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.

Target Information

The second major step in the catabolism of branched-chain amino acids (isoleucine, leucine, and valine) is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of three catalytic components: E1 (decarboxylase), E2 (transacylase), and E3 (dehydrogenase).
The complex also includes two regulatory enzymes: a kinase (BCKDK) and a phosphorylase. The ubiquitously expressed kinase contains one histidine kinase domain.
Maple syrup urine disease (MSUD) results from defects in the catabolic pathway of these amino acids, leading to accumulation of toxic metabolites and neurodegeneration in infants.

⚠ 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, visit www.P65Warnings.ca.gov.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

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