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BCKDHA Recombinant Protein

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS203795

BCKDHA (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain), also known as 2-oxoisovalerate dehydrogenase subunit alpha, belongs to the BCKDHA family. The branched-chain alpha-keto acid dehydrogenase complex is an innter mitochondrial… More details

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Product Documentation
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Sample Manual Insert View PDF
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Testing Data View PDF
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Certificate of Analysis (COA) View PDF
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QC Report View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Protocol View PDF

Product Overview

Online Datasheet in PDF
Product Name
BCKDHA, Recombinant Protein
Full Product Name
BCKDHA, 46-445aa, Human, His tag, E Coli
Product Synonym Names
2-oxoisovalerate dehydrogenase subunit alpha; BCKDE1A; BCKDH E1-alpha; MSu; MSuD1; OVD1A
Sequence
MGSSHHHHHH SSGLVPRGSH MSSLDDKPQF PGASAEFIDK LEFIQPNVIS GIPIYRVMDR QGQIINPSED PHLPKEKVLK LYKSMTLLNT MDRILYESQR QGRISFYMTN YGEEGTHVGS AAALDNTDLV FGQYREAGVL MYRDYPLELF MAQCYGNISD LGKGRQMPVH YGCKERHFVT ISSPLATQIP QAVGAAYAAK RANANRVVIC YFGEGAASEG DAHAGFNFAA TLECPIIFFC RNNGYAISTP TSEQYRGDGI AARGPGYGIM SIRVDGNDVF AVYNATKEAR RRAVAENQPF LIEAMTYRIG HHSTSDDSSA YRSVDEVNYW DKQDHPISRL RHYLLSQGWW DEEQEKAWRK QSRRKVMEAF EQAERKPKPN PNLLFSDVYQ EMPAQLRKQQ ESLARHLQTY GEHYPLDHFD K
Host
E Coli
Form/Format
Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 5mM DTT, 30% glycerol, 0.2M NaCl
Purity/Purification
> 80% by SDS-PAGE
Concentration
0.25 mg/ml (determined by Bradford assay) (lot specific)
Preparation and Storage
Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.
Molecular Weight
47.8kDa (421aa) confirmed by MALDI-TOF
OMIM
Z14093 mRNA
3D Structure
ModBase 3D Structure for P12694
Antigen Species
Human
Tag Information
His-tag

Product Categories/Family for BCKDHA recombinant protein

Applications Tested/Suitable for BCKDHA recombinant protein
SDS-PAGE

Product References and Citations for BCKDHA recombinant protein

Nobukuni Y., et al. (1993) Biochim. Biophys. Acta 1225:64-70; Funchal, C., et al. (2005) Metab. Brain Dis. 20: 205-217

Other Notes

Small volumes of BCKDHA recombinant protein 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 BCKDHA purchase

MBS203795 is a recombinant protein targeting BCKDHA. Also known as BCKDHA, 46-445aa, Human, His tag, E Coli. Host: E Coli.

Additional Details

NCBI/Uniprot data below describe general gene information for BCKDHA. It may not necessarily be applicable to this product.

NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
GenBank Nucleotide #
NCBI Official Full Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Official Symbol
BCKDHA
NCBI Official Synonym Symbols
MSU; MSUD1; OVD1A; BCKDE1A
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
UniProt Synonym Gene Names
BCKDE1A; BCKDH E1-alpha
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

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]
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UniProt Comments for BCKDHA

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

Precautions

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.

Disclaimer

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.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. 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, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

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