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Rat Bckdha cDNA Clone(NM_012782.1)

For research use only. Not intended for any clinical use.

Cat. No. :   CDFR010584

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Gene Information

Cat. No. CDFR010584
Description NativeORF clone of Rattus norvegicus branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha) as transfection-ready DNA NM_012782.1
Product Type cDAN Clone
Gene Abbr Bckdha
Species Rat
Vector pCMV6Entry
Target Gene BCKDHA
Background The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]
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