BCKDHB rabbit monoclonal antibody

Catalog # H00000594-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 BCKDHB peptide using ARM Technology.

    Immunogen

    A synthetic peptide of human BCKDHB 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 BCKDHB 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 — BCKDHB

    Entrez GeneID

    594

    GeneBank Accession#

    BCKDHB

    Gene Name

    BCKDHB

    Gene Alias

    E1B, FLJ17880, dJ279A18.1

    Gene Description

    branched chain keto acid dehydrogenase E1, beta polypeptide

    Omim ID

    248600 248611

    Gene Ontology

    Hyperlink

    Gene Summary

    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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq

    Other Designations

    2-oxoisovalerate dehydrogenase beta subunit|E1b-beta subunit of the branched-chain complex|OTTHUMP00000018048|OTTHUMP00000018049|branched chain alpha-ketoacid dehydrogenase E1-beta subunit|branched chain keto acid dehydrogenase E1 beta polypeptide|branche

  • 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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