Clone Name: HL2434
Light Chain: Kappa
Application Note: WB: 1:500-1:3000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
Calculated MW: 50
Form: Liquid
Buffer (with preservative): PBS, no preservative.
Concentration: 1 mg/ml (Please refer to the vial label for the specific concentration.)
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]
Uniprot ID: P12694
Antigen Species: Human
Immunogen: Recombinant fragment of human BCKDHA.
Purification: Affinity purified by Protein A.
Conjugation: Unconjugated
Full Name: branched chain keto acid dehydrogenase E1, alpha polypeptide
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