BCKDHA polyclonal antibody
Product Specifications
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.
CAS Number
9007-83-4
Structure Composition
1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Product Name Alternative
BCKDHA; BCKDE1A; MSU; MSUD1; OVD1A
Swiss Prot
P12694
Reactivity
Human, Mouse, Rat
Immunogen
Recombinant fusion protein of human BCKDHA (NP_000700.1) .
Conjugation
Unconjugated
Applications
WB, IF/ICC
Dilution
WB,1:500 - 1:2000|IF/ICC,1:50 - 1:200
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Modification
Unmodified
Molecular Weight
50kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
BCKDHA polyclonal antibody detects endogenous levels of BCKDHA protein.
Applications Notes
Western blot analysis of various lysates, using BCKDHA pAb at 1:2000 dilution.
Host or Source
Rabbit
Available Sizes
Curated Selection
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