BCKDHA rabbit monoclonal antibody

Catalog # H00000593-K

Size

Price

Stock

Quantity

Size:100 ug x up to 3
Price: -
Stock:
made to order, 8 months
abnova-minus
abnova-plus

* The price is valid only in USA. Please select country.

Contact Info
  • +1-909-264-1399
    +1-909-992-0619
    Toll Free : +1-877-853-6098
  • +1-909-992-3401
  • Specification

    Product Description

    Rabbit monoclonal antibody raised against a human BCKDHA peptide using ARM Technology.

    Immunogen

    A synthetic peptide of human BCKDHA is used for rabbit immunization.
    Customer or Abnova will decide on the preferred peptide sequence.

    Host

    Rabbit

    Library Construction

    Non-fusion antibody library from rabbit spleen (ARM Technology).

    Expression

    Overexpression vector and transfection into 293H cell line.

    Reactivity

    Human

    Purification

    Protein A

    Isotype

    IgG

    Quality Control Testing

    Antibody reactive against human BCKDHA peptide by ELISA and mammalian transfected lysate by Western Blot.

    Storage Buffer

    In 1x PBS, pH 7.4

    Storage Instruction

    Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.

    Deliverable

    Up to three rabbit IgG clones of 100 ug each will be delivered to customer.

    Note

    1. Customer may provide cell or tissue lysate for antibody screening.
    2. Rabbit monoclonal antibody generated by ARM technology is amenable to antibody engineering including F(ab)2, IgG, scFv and different Fc and non-Fc conjugates per customer request.

  • Applications

    Western Blot (Transfected lysate)

    ELISA

  • Gene Info — BCKDHA

    Entrez GeneID

    593

    GeneBank Accession#

    BCKDHA

    Gene Name

    BCKDHA

    Gene Alias

    BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A

    Gene Description

    branched chain keto acid dehydrogenase E1, alpha polypeptide

    Omim ID

    248600 608348

    Gene Ontology

    Hyperlink

    Gene Summary

    The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene

    Other Designations

    2-oxoisovalerate dehydrogenase (lipoamide)|branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit|maple syrup urine disease

  • Interactome
  • Pathway
  • Disease
Contact Info
  • +1-909-264-1399
    +1-909-992-0619
    Toll Free : +1-877-853-6098
  • +1-909-992-3401
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