BCKDHB Antibody

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) . This gene encodes the E1 beta subunit, and mutations therein 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.

Product Specifications

CAS Number

9007-83-4

Specifications

Western blot: 1:500-1:2000, Immunohistochemistry (FFPE) : 1:25, Immunofluorescence: 1:25, Flow cytometry: 1:25 (1x10e6 cells)

UniProt

P21953

Host

Rabbit

Reactivity

Human

Immunogen

A portion of amino acids 41-70 from the human protein were used as the immunogen for the BCKDHB antibody.

Clonality

Polyclonal

Isotype

Applications

WB, IHC-P, IF, FACS

Purity

Antigen affinity purified

Format

Purified

Buffer

In 1X PBS, pH 7.4, with 0.09% sodium azide

Reconstitution

Aliquot the BCKDHB antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Limitations

This BCKDHB antibody is available for research use only.

Storage Conditions

Aliquot the BCKDHB antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.

Formulation

In 1X PBS, pH 7.4, with 0.09% sodium azide

Applications Notes

The stated application concentrations are suggested starting points. Titration of the BCKDHB antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Location

Cytoplasmic

Image Legend

Western blot testing of human 1) HeLa, 2) 293T and 3) Jurkat cell lysate with BCKDHB antibody. Predicted molecular weight: ~43 kDa, observed molecular weight: ~35 kDa.