BCKDHA Rabbit Polyclonal Antibody
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Specifications
Product Data | |
Applications | WB |
Recommended Dilution | WB,1:500 - 1:2000 |
Reactivities | Human, Mouse, Rat |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
Formulation | Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |
Concentration | lot specific |
Purification | Affinity purification |
Conjugation | Unconjugated |
Stability | Shelf life: one year from despatch. |
Predicted Protein Size | 50kDa |
Gene Name | branched chain keto acid dehydrogenase E1 subunit alpha |
Database Link | |
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. |
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