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BCKDHA Protein

Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial, BCKDE1A

Catalog No. Product Name Size List Price (US$) Quantity
BP000561-ENZ-090 Recombinant Human Branched Chain keto Acid Dehydrogenase E1 Alpha 5 ug 160.00
BP000561-ENZ-090 Recombinant Human Branched Chain keto Acid Dehydrogenase E1 Alpha 50 ug 1200.00
Description

Introduction
The BCKD (branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA.

BP000561-ENZ-090: Recombinant Human Branched Chain keto Acid Dehydrogenase E1 Alpha

Source: E. coli-derived.
Predicted molecular mass: 47.8 kDa.
Purity > 80%, by SDS-PAGE.
Formulation: The BCKDHA solution (0.25 mg/ml) contains 20 mM Tris-HCl buffer (pH 8.0), 5 mM DTT, 30% glycerol and 0.2 M NaCl.

Shipping: The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70°C as supplied.
1 month, 2 to 8°C under sterile conditions after reconstitution.
3 months, -20 to -70°C under sterile conditions after reconstitution.

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