BCKDHB polyclonal antibody
Product Specifications
Background
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.
CAS Number
9007-83-4
Structure Composition
1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Product Name Alternative
BCKDHB; BCKDE1B; BCKDH E1-beta; E1B; BCKDHE1-beta
Swiss Prot
P21953
Reactivity
Mouse
Immunogen
Recombinant fusion protein of human BCKDHB (NP_898871.1) .
Conjugation
Unconjugated
Applications
WB
Dilution
WB,1:500 - 1:2000
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
Unmodification
Molecular Weight
43kDa
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
Unmodification
Host or Source
Rabbit
Available Sizes
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