BCKDHB Monoclonal Antibody

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.

Description
BCKDHB Monoclonal Antibody. Unconjugated. Raised in: Rabbit.

Formulation
Buffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.

Specificity
Human, Mouse, Rat

Isotype
IgG

Uniprot ID
P21953

Purification
Affinity Purified

Immunogen
Recombinant protein (or fragment). Please contact EpigenTek for more information.

Storage
Store at -20°C. Avoid freeze / thaw cycles.

Alternative Names
E1B; BCKDE1B; BCKDH E1-beta; BCKDHB

Application
WB, ELISA; Recommended dilution: WB, 1:2000 - 1:12000; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.