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BCKDHA elisa kit :: Guinea Pig 2-oxoisovalee dehydrogenase subunit alpha, mitochondrial (BCKDHA) ELISA Kit

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Catalog # MBS7211022
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BCKDHA elisa kit
Product Name

2-oxoisovalee dehydrogenase subunit alpha, mitochondrial (BCKDHA), ELISA Kit

Full Product Name

Guinea pig 2-oxoisovalee dehydrogenase subunit alpha, mitochondrial (BCKDHA) ELISA Kit

Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Request for Current Manual Insert
Species Reactivity
Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
Preparation and Storage
Store all reagents at 2-8 degree C.
Sample Preparation
We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team
Product Note
Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.
Other Notes
Small volumes of BCKDHA elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Searchable Terms for BCKDHApurchase
MBS7211022 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 2-oxoisovalee dehydrogenase subunit alpha, mitochondrial (BCKDHA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing BCKDHA. The ELISA analytical biochemical technique of the MBS7211022 kit is based on BCKDHA antibody-BCKDHA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect BCKDHA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, BCKDHA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
NCBI/Uniprot data below describe general gene information for BCKDHA. It may not necessarily be applicable to this product.
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
50,471 Da
NCBI Official Full Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 2
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Official Symbol
BCKDHA  [Similar Products]
NCBI Official Synonym Symbols
  [Similar Products]
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDH E1-alpha; 2-oxoisovalerate dehydrogenase (lipoamide); branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
UniProt Protein Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
UniProt Synonym Protein Names
Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha
UniProt Gene Name
BCKDHA  [Similar Products]
UniProt Synonym Gene Names
BCKDE1A; BCKDH E1-alpha  [Similar Products]
UniProt Entry Name
NCBI Summary for BCKDHA
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.[provided by RefSeq, Sep 2009]
UniProt Comments for BCKDHA
BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation

Protein type: Oxidoreductase; Lyase; EC; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 19q13.1-q13.2

Cellular Component: mitochondrion; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix

Molecular Function: protein binding; alpha-ketoacid dehydrogenase activity; metal ion binding; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity

Biological Process: branched chain family amino acid catabolic process

Disease: Maple Syrup Urine Disease
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

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