BCKDHB Polyclonal Antibody

Catalog Number:

27467

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P21953
NCBI Gene ID:594

Form of Antibody:

Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.

Storage/Stability:

Immunogen:

Recombinant fusion protein of human BCKDHB (NP_898871.1).

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB

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.

References:

appl_detail:

WB 1:500 - 1:2000

Datasheet PDF