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

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) .