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BCKDHA recombinant protein :: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA) Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS952592 BCKDHA recombinant protein
Unit / Price
0.05 mg (E-Coli)  /  $815 +1 FREE 8GB USB
0.05 mg (Baculovirus)  /  $1,050 +1 FREE 8GB USB
0.2 mg (E-Coli)  /  $1,090 +1 FREE 8GB USB
0.5 mg (E-Coli)  /  $1,200 +1 FREE 8GB USB
0.2 mg (Yeast)  /  $1,235 +1 FREE 8GB USB
0.05 mg (Mammalian-Cell)  /  $1,295 +1 FREE 8GB USB
0.1 mg (Baculovirus)  /  $1,330 +1 FREE 8GB USB
0.5 mg (Yeast)  /  $1,400 +1 FREE 8GB USB
1 mg (E-Coli)  /  $1,705 +2 FREE 8GB USB
0.5 mg (Baculovirus)  /  $1,910 +2 FREE 8GB USB
0.1 mg (Mammalian-Cell)  /  $1,960 +2 FREE 8GB USB
1 mg (Yeast)  /  $2,125 +2 FREE 8GB USB
1 mg (Baculovirus)  /  $2,520 +3 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA), Recombinant Protein

 Also Known As   

Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA)

 Product Gene Name   

BCKDHA recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence Positions    46-445aa, Full Length of Mature Protein
 Sequence    SSLDDKPQFP GASAEFIDKL EFIQPNVISG IPIYRVMDRQ GQIINPSEDP HLPKEKVLKL YKSMTLLNTM DRILYESQRQ GRISFYMTNY GEEGTHVGSA AALDNTDLVF GQYREAGVLM YRDYPLELFM AQCYGNISDL GKGRQMPVHY GCKERHFVTI SSPLATQIPQ AVGAAYAAKR ANANRVVICY FGEGAASEGD AHAGFNFAAT LECPIIFFCR NNGYAISTPT SEQYRGDGIA ARGPGYGIMS IRVDGNDVFA VYNATKEARR RAVAENQPFL IEAMTYRIGH HSTSDDSSAY RSVDEVNYWD KQDHPISRLR HYLLSQGWWD EEQEKAWRKQ SRRKVMEAFE QAERKPKPNP NLLFSDVYQE MPAQLRKQQE SLARHLQTYG EHYPLDHFDK
 OMIM    248600
 3D Structure    ModBase 3D Structure for P12694
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 Host    E Coli or Yeast or Baculovirus or Mammalian Cell
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 Purity/Purification    >85% (SDS-PAGE) (lot specific)
 Form/Format    Liquid containing glycerol
 Tag Information    This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.
 Sterility    Sterile filter available upon request.
 Endotoxin    Low endotoxin available upon request.
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 Species    Human
 Storage Buffer    Tris-based buffer, 50% glycerol
 Tag Information    Tag type will be determined during the manufacturing process
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 Preparation and Storage    Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 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.
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NCBI/Uniprot data below describe general gene information for BCKDHA. It may not necessarily be applicable to this product.
 NCBI GI #    11386135
 NCBI GeneID    593
 NCBI Accession #    NP_000700.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000709.3 [Other Products]
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 UniProt Primary Accession #    P12694 [Other Products]
 UniProt Secondary Accession #    Q16034; Q16472; B4DP47; E7EW46 [Other Products]
 UniProt Related Accession #    P12694 [Other Products]
 Molecular Weight    50,790 Da
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 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 [Similar Products]
 NCBI Official Synonym Symbols   
MSU; MSUD1; OVD1A; BCKDE1A
[Similar Products]
 NCBI Protein Information    2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
 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]
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 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).
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 Research Articles on BCKDHA    1. we identified seven novel genetic variants: p.Leu549Pro, p.Glu564*, p.Leu641Pro in MUT, p.Tyr206Cys in PCCB, p.His194Arg, p.Val298Met in BCKDHA and p.Glu286_Met290del in BCKDHB gene. In silico and/or eukaryotic expression studies confirmed pathogenic effect of all novel genetic variants
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 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.
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Products associated with BCKDHA recombinant proteinPathways associated with BCKDHA recombinant protein
 Reference Product  PubMed Publications
 BCKDHB recombinant protein  >22 publications with BCKDHA and BCKDHB
 DBT recombinant protein  >21 publications with BCKDHA and DBT
 DLD recombinant protein  >4 publications with BCKDHA and DLD
 IVD recombinant protein  >1 publications with BCKDHA and IVD
 BCAT2 recombinant protein  >1 publications with BCKDHA and BCAT2
 Products by Pathway  Pathway Diagram
 2-oxobutanoate Degradation Pathway antibodies  2-oxobutanoate Degradation Pathway Diagram
 2-oxobutanoate Degradation I Pathway antibodies  2-oxobutanoate Degradation I Pathway Diagram
 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway antibodies  2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway Diagram
 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway antibodies  2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway Diagram
 Branched-chain Amino Acid Catabolism Pathway antibodies  Branched-chain Amino Acid Catabolism Pathway Diagram
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
Diseases associated with BCKDHA recombinant proteinOrgans/Tissues associated with BCKDHA recombinant protein
 Disease Name  Pubmed Publications
 Maple Syrup Urine Disease Antibodies  >28 publications with BCKDHA and Maple Syrup Urine Disease
 Edema Antibodies  >1 publications with BCKDHA and Edema
 Liver Cirrhosis Antibodies  >1 publications with BCKDHA and Liver Cirrhosis
 Liver Failure Antibodies  >1 publications with BCKDHA and Liver Failure
 Coma Antibodies  >1 publications with BCKDHA and Coma
 Fibrosis Antibodies  >1 publications with BCKDHA and Fibrosis
 Fatty Liver Antibodies  >1 publications with BCKDHA and Fatty Liver
 Organ/Tissue Name  Pubmed Publications
 Liver Antibodies  >6 publications with BCKDHA and Liver
 Blood Antibodies  >6 publications with BCKDHA and Blood
 Muscle Antibodies  >4 publications with BCKDHA and Muscle
 Heart Antibodies  >2 publications with BCKDHA and Heart
 Adipose Tissue Antibodies  >2 publications with BCKDHA and Adipose Tissue
 Kidney Antibodies  >2 publications with BCKDHA and Kidney
 Intestine Antibodies  >2 publications with BCKDHA and Intestine
 Lung Antibodies  >2 publications with BCKDHA and Lung
 Connective Tissue Antibodies  >1 publications with BCKDHA and Connective Tissue
 Ovary Antibodies  >1 publications with BCKDHA and Ovary
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