Thermo Fisher Scientific BCKDHA Recombinant Rabbit Monoclonal Antibody (HL2434)

Applications

Western Blot (WB)

• Tested Dilution: 1:500–1:3,000

Immunohistochemistry (Paraffin) (IHC (P))

• Tested Dilution: Assay-dependent

Product Specifications

Target Information

The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine.
This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998).

The BCKD complex consists of three catalytic components:

• A heterotetrameric (α2β2) branched-chain alpha-keto acid decarboxylase (E1)
• A homo-24-meric dihydrolipoyl transacylase (E2)
• A homodimeric dihydrolipoamide dehydrogenase (E3)

E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene encodes the beta subunit of E1. The complex also contains two regulatory enzymes, a kinase and a phosphorylase.

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