Skip to product information
1 of 1

NSJ Bioreagents

SKU:F54271-0.08ML

BCKDHB Antibody

BCKDHB Antibody

Regular price $205.00 USD
Regular price Sale price $205.00 USD
Sale Sold out
Shipping calculated at checkout.
Size

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.

Specifications

Family Primary antibody
Formulation In 1X PBS, pH 7.4, with 0.09% sodium azide
Format Purified
Host Animal Rabbit
Clonality Polyclonal (rabbit origin)
Isotype Rabbit Ig
Species Reactivity Human
Application WB, IHC-P, IF, FACS
Application Details Western blot: 1:500-1:2000,Immunohistochemistry (FFPE): 1:25,Immunofluorescence: 1:25,Flow cytometry: 1:25 (1x10e6 cells)
Application Note 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.
Localization Cytoplasmic
Immunogen A portion of amino acids 41-70 from the human protein were used as the immunogen for the BCKDHB antibody.
Purity Antigen affinity purified
Storage Aliquot the BCKDHB antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Limitation This BCKDHB antibody is available for research use only.
Uniprot # P21953
Status Available
PDF Link https://www.nsjbio.com/tds-pdf/bckdhb-antibody-f54271
Title BCKDHB Antibody
Description 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.
View full details