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Product Name
BCKDHA Rabbit pAb
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Gene ID
593
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SwissProt ID
P12694
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Gene Name
BCKDHA
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Alternative Names
MSU; MSUD1; OVD1A; BCKDE1A; BCKDHA
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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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Research Field
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Product Categories
Primary antibody
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Host
Rabbit
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Reactivity
Human, Mouse, Rat
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Application
WB, ICC/IF
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Dilution Ratio
WB: 1/500-1/1000 IF: 1/50-1/200
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Molecular Weight
Calculated MW:50kDa
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Clonality
Polyclonal Antibody
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Clonality No.
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Isotype
IgG
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Immunogen
Recombinant fusion protein
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Purification
Affinity Purified
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Conjugation
Unconjugated
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Modification
Unmodified
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Form
Liquid
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Buffer System
Liquid in PBS with 0.02% sodium azide,50% glycerol,pH7.3.
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Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.