BCKDHA rabbit monoclonal antibody
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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
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Gene Info — BCKDHA
Entrez GeneID
593GeneBank Accession#
BCKDHAGene Name
BCKDHA
Gene Alias
BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A
Gene Description
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Ontology
HyperlinkGene 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
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