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Recommended positive controls: A549, HeLa, HepG2.
Predicted reactivity: Mouse (95%), Rat (95%), Pig (94%), Sheep (95%), Chimpanzee (98%), Bovine (94%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2), and 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 2 regulatory enzymes, a kinase and a phosphorylase.
⚠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: 2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; branched chain keto acid dehydrogenase E1 alpha protein; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
Gene Aliases: BCKDE1A; BCKDHA; MSU; MSUD1; OVD1A
UniProt ID: (Human) P12694
Entrez Gene ID: (Human) 593
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