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

Scan QR to view Datasheet Catalog #    MBS7217582 BCKDHA elisa kit
Unit / Price
48-Strip-Wells  /  $455 +1 FREE 8GB USB
96-Strip-Wells  /  $660 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,925 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,490 +6 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-oxoisovalee dehydrogenase subunit alpha, mitochondrial (BCKDHA), ELISA Kit

 Also Known As   

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

 Product Gene Name   

BCKDHA elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Request for Current Manual Insert    Request Current Manual
 OMIM    608348
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 Species Reactivity    Mouse
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 Samples    Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
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 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 BCKDHA purchase    MBS7217582 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 MBS7217582 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 such as Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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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 #    258645172
 NCBI GeneID    593
 NCBI Accession #    NP_001158255.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001164783.1 [Other Products]
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 UniProt Secondary Accession #    Q16034; Q16472 [Other Products]
 UniProt Related Accession #    P12694 [Other Products]
 Molecular Weight    50,471 Da
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 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   
MSU; MSUD1; OVD1A; BCKDE1A
[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    ODBA_HUMAN
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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    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: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; EC 1.2.4.4; Lyase; Mitochondrial

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
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 Research Articles on BCKDHA    1. Data from infant/her heterozygous parents (first cousins) suggest homozygous mutation (S144I) in BCKDHA can result in maple syrup urine disease (IA); molecular modeling suggests this missense mutation in exon 4 affects protein stability. [CASE STUDY]
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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 elisa kitPathways associated with BCKDHA elisa kit
 Reference Product  PubMed Publications
 BCKDHB elisa kit  >20 publications with BCKDHA and BCKDHB
 DBT elisa kit  >19 publications with BCKDHA and DBT
 DLD elisa kit  >2 publications with BCKDHA and DLD
 BCAT2 elisa kit  >1 publications with BCKDHA and BCAT2
 Products by Pathway  Pathway Diagram
 2-oxobutanoate Degradation I Pathway antibodies  2-oxobutanoate Degradation I 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 elisa kitOrgans/Tissues associated with BCKDHA elisa kit
 Disease Name  Pubmed Publications
 Maple Syrup Urine Disease Antibodies  >26 publications with BCKDHA and Maple Syrup Urine Disease
 Fibrosis Antibodies  >1 publications with BCKDHA and Fibrosis
 Liver Cirrhosis Antibodies  >1 publications with BCKDHA and Liver Cirrhosis
 Edema Antibodies  >1 publications with BCKDHA and Edema
 Liver Failure Antibodies  >1 publications with BCKDHA and Liver Failure
 Coma Antibodies  >1 publications with BCKDHA and Coma
 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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