branched chain keto acid dehydrogenase E1, alpha polypeptide, Mouse, Polyclonal Antibody, Abnova
Mouse polyclonal antibody raised against a partial recombinant BCKDHA.
Manufacturer: Abnova Corporation H00000593A01
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 geneSequence: SVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
|branched chain keto acid dehydrogenase E1, alpha polypeptide|
|Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.|
|ELISA, Western Blot|
|Mouse polyclonal antibody raised against a partial recombinant BCKDHA.|
|BCKDHA (NP_000700, 347 a.a. ~ 445 a.a) partial recombinant protein with GST tag.|
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