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Rat Bckdha ORF Clone(NM_012782.1)

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

Cat. No. :   CDCR377642

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Product Information

Gene Information

Cat. No. CDCR377642
Product Type ORF Clone
Gene Abbr Bckdha
Species Rat
Tag C-terminal Myc-DDK
ORF Size 1341 bp
Vector pCMV6-Entry
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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