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BCKDHA Rabbit pAb

CNA16774S
Antibody
100 µl

THIS ITEM HAS BEEN DISCONTINUED

DATA SHEET

TargetBCKDHA
Product TypeAntibody
ApplicationWB
ClonalityPolyclonal
IsotypeIgG
ImmunogenRecombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1).
Host SpeciesRabbit
Species ReactivityHuman, Mouse, Rat
FormulationPBS with 0.02% sodium azide,50% glycerol
Research AreaCancer, Immunology
Description/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.

ProtocolsWB
Molecular Weight50kDa
PurityAffinity purification
Regulatory StatementFor Research Use Only. Not for use in diagnostic procedures.
CNA16774S
Antibody
100 µl

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