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anti-BCKD antibody :: Rabbit BCKD Antibody

Scan QR to view Datasheet Catalog #    MBS000184 anti-BCKD antibody
Unit / Price
0.05 mL  /  $270 +1 FREE 8GB USB
0.1 mL  /  $445 +1 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   

BCKD, Antibody

 Also Known As   

Rabbit BCKD Antibody

 Product Gene Name   

anti-BCKD antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 16; NC_000016.9 (31119662..31124112). Location: 16p11.2
 OMIM    614901
 3D Structure    ModBase 3D Structure for O14874
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 Host    Rabbit
 Species Reactivity    Human, mouse, rat
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 Form/Format    Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol
 Concentration    1 mg/ml (lot specific)
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 Other Notes    Small volumes of anti-BCKD antibody 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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 Applications Tested/Suitable for anti-BCKD antibody   

ELISA (EIA), Immunofluorescence (IF), Western Blot (WB)

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NCBI/Uniprot data below describe general gene information for BCKD. It may not necessarily be applicable to this product.
 NCBI GI #    171906593
 NCBI GeneID    10295
 NCBI Accession #    NP_001116429.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001122957.2 [Other Products]
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 UniProt Primary Accession #    O14874 [Other Products]
 UniProt Secondary Accession #    Q6FGL4; Q96IN5 [Other Products]
 UniProt Related Accession #    O14874 [Other Products]
 Molecular Weight    46,360 Da
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 NCBI Official Full Name    [3-methyl-2-oxobutanoate dehydrogenase
 NCBI Official Synonym Full Names    branched chain ketoacid dehydrogenase kinase
 NCBI Official Symbol    BCKDK [Similar Products]
 NCBI Official Synonym Symbols   
BDK; BCKDKD
[Similar Products]
 NCBI Protein Information    [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKDHKIN; BCKD-kinase; branched chain alpha-ketoacid dehydrogenase kinase
 UniProt Protein Name    [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
 UniProt Synonym Protein Names   
Branched-chain alpha-ketoacid dehydrogenase kinase
 UniProt Gene Name    BCKDK [Similar Products]
 UniProt Synonym Gene Names    BCKD-kinase; BCKDHKIN [Similar Products]
 UniProt Entry Name    BCKD_HUMAN
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 NCBI Summary for BCKD    The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]
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 UniProt Comments for BCKD    BCKDK: an atypical protein kinase associated with the mitochondrial matrix. Contains a HATPase_c domain, found in several ATP-binding proteins including protein histidine kinases (PHKs), PHDKs, DNA gyrase B, topoisomerases, heat shock proteins, and DNA mismatch repair proteins. Unlike PHKs, BCK dimerizes through direct interaction of two opposing nucleotide-binding domains. Phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Inactivating mutations have been discovered in BCKDK in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation.

Protein type: Protein kinase, atypical; Kinase, protein; Mitochondrial; EC 2.7.11.4; ATYPICAL group; PDHK family

Chromosomal Location of Human Ortholog: 16p11.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrion

Molecular Function: protein serine/threonine kinase activity; protein binding; [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; kinase activity; ATP binding

Biological Process: branched chain family amino acid catabolic process; amino acid catabolic process; phosphorylation; protein amino acid phosphorylation

Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency
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 Research Articles on BCKD    1. Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
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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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Diseases associated with anti-BCKD antibodyOrgans/Tissues associated with anti-BCKD antibody
 Disease Name  Pubmed Publications
 Neurodegenerative Diseases Antibodies  >1 publications with BCKD and Neurodegenerative Diseases
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >3 publications with BCKD and Brain
 Blood Antibodies  >2 publications with BCKD and Blood
 Liver Antibodies  >2 publications with BCKD and Liver
 Muscle Antibodies  >1 publications with BCKD and Muscle
 Kidney Antibodies  >1 publications with BCKD and Kidney
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