Thermo Fisher Scientific BCKDK Polyclonal Antibody

Applications

Western Blot (WB)

• Tested Dilution: 1:500–1:3,000

Immunocytochemistry (ICC/IF)

• Tested Dilution: 1:100–1:500

Product Specifications

Product Specific Information

The antibody detects endogenous levels of total BCKDK protein.

Target Information

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 three catalytic components:

• Branched-chain alpha-keto acid decarboxylase (E1)
• Dihydrolipoyl transacylase (E2)
• Dihydrolipoamide dehydrogenase (E3)

The complex also contains two enzymes that regulate its activity state: a kinase (BCKDK) and a phosphorylase. The ubiquitously expressed kinase contains one histidine kinase domain.
Maple syrup urine disease (MSUD) arises from a defect in this catabolic pathway, leading to accumulation of branched-chain amino acids and their keto acids, resulting in encephalopathy and progressive neurodegeneration in untreated infants.

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