Overview

  • Product name
  • Description
    Rabbit polyclonal to BCKDHB
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide conjugated to KLH, corresponding to a region within N terminal amino acids 44-74 of Human BCKDHB (NP_000047.1).

  • Positive control
    • HeLa cell line lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab107398 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/500. Predicted molecular weight: 43 kDa.

Target

  • Function
    The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
  • Involvement in disease
    Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B) [MIM:248600]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt
  • Database links
  • Alternative names
    • 2 oxoisovalerate dehydrogenase subunit beta mitochondrial antibody
    • 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial antibody
    • BCKDE1B antibody
    • BCKDH E1-beta antibody
    • BCKDHB antibody
    • Branched chain alpha ketoacid dehydrogenase E1 beta subunit antibody
    • Branched chain keto acid dehydrogenase E1 beta polypeptide antibody
    • Branched-chain alpha-keto acid dehydrogenase E1 component beta chain antibody
    • dJ279A18.1 antibody
    • E1B antibody
    • E1b beta subunit of the branched chain complex antibody
    • FLJ17880 antibody
    • ODBB_HUMAN antibody
    • RP1-279A18.1 antibody
    see all

Images

  • Anti-BCKDHB antibody (ab107398) at 1/100 dilution + HeLa cell line lysate at 35 µg

    Predicted band size : 43 kDa

References

ab107398 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab107398.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up