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BCKDHA Human - Recombinant Human Branched Chain keto Acid Dehydrogenase E1, Alpha (1ug)

BCKDHA Human - Recombinant Human Branched Chain keto Acid Dehydrogenase E1, Alpha (1ug)

Product Introduction: Branched-chain ?-keto acid dehydrogenase E1 component ? chain (BCKDHA) is a member of the BCKDHA family. 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.
Product Description: BCKDHA Human Recombinant fused with a 21 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 421 amino acids (46-445 a.a.) and having a molecular mass of 47.8kDa. The BCKDHA is purified by proprietary chromatographic techniques.

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SPECIFICATIONS

Size

1ug

Format

Sterile Filtered colorless solution.

Presku

OOPA00107

Alternative Names

2-oxoisovalerate dehydrogenase subunit alpha mitochondrial; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha; BCKDHA; MSU; MSUD1; OVD1A; FLJ45695.

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