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NDUFV1 elisa kit :: Human NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa ELISA Kit

Scan QR to view Datasheet Catalog #    MBS9318690 NDUFV1 elisa kit
Unit / Price
48-Strip-Wells  /  $435 +1 FREE 8GB USB
96-Strip-Wells  /  $600 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,650 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,205 +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   

NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa (NDUFV1), ELISA Kit

 Also Known As   

Human NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial, NDUFV1 ELISA Kit

 Product Synonym Names    Human NADH dehydrogenase [ubiquinone] flavoprotein 1; mitochondrial (NDUFV1) ELISA kit; CI51KD; FLJ59059; UQOR1; NADH dehydrogenase ubiquinone flavoprotein 1; NADH-ubiquinone oxidoreductase; complex I; mitochondrial respiratory chain; NADH dehydrogenase (ubiquinone) flavoprotein 1; 51kDa
 Product Gene Name   

NDUFV1 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    256000
 3D Structure    ModBase 3D Structure for P49821
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 Species Reactivity    Human
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 Preparation and Storage    Store all reagents at 2-8 degree C
 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 NDUFV1 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 NDUFV1 purchase    MBS9318690 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa (NDUFV1) 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 NDUFV1. The ELISA analytical biochemical technique of the MBS9318690 kit is based on NDUFV1 antibody-NDUFV1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect NDUFV1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, NDUFV1. 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(%).
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NCBI/Uniprot data below describe general gene information for NDUFV1. It may not necessarily be applicable to this product.
 NCBI GI #    260656005
 NCBI GeneID    4723
 NCBI Accession #    NP_001159574.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001166102.1 [Other Products]
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 UniProt Primary Accession #    P49821 [Other Products]
 UniProt Secondary Accession #    O60924; O60940; Q16104; Q6IBR3; Q96BF8; Q96HS7 [Other Products]
 UniProt Related Accession #    P49821 [Other Products]
 Molecular Weight    50,817 Da
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 NCBI Official Full Name    NADH dehydrogenase
 NCBI Official Synonym Full Names    NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
 NCBI Official Symbol    NDUFV1 [Similar Products]
 NCBI Official Synonym Symbols   
UQOR1; CI-51K; CI51KD
[Similar Products]
 NCBI Protein Information    NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; complex I 51kDa subunit; complex I 51 kda subunit; complex I, mitochondrial respiratory chain; NADH-ubiquinone oxidoreductase 51 kDa subunit; mitochondrial NADH dehydrogenase ubiquinone flavoprotein 1; mitochondrial NADH:ubiquinone oxidoreductase 51 kda subunit
 UniProt Protein Name    NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
 UniProt Synonym Protein Names   
Complex I-51kD; CI-51kD; NADH dehydrogenase flavoprotein 1; NADH-ubiquinone oxidoreductase 51 kDa subunit
 UniProt Gene Name    NDUFV1 [Similar Products]
 UniProt Synonym Gene Names    UQOR1; CI-51kD [Similar Products]
 UniProt Entry Name    NDUV1_HUMAN
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 NCBI Summary for NDUFV1    The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
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 UniProt Comments for NDUFV1    NDUFV1: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Defects in NDUFV1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I 51 kDa subunit family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.6.5.3; Oxidoreductase; EC 1.6.99.3; Energy Metabolism - oxidative phosphorylation; Mitochondrial

Chromosomal Location of Human Ortholog: 11q13

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: NADH dehydrogenase (ubiquinone) activity; FMN binding; 4 iron, 4 sulfur cluster binding; metal ion binding; NAD binding

Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone

Disease: Mitochondrial Complex I Deficiency
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 Research Articles on NDUFV1    1. observed 2 consanguinous siblings with early-onset encephalopathy, medulla, brainstem and mesencephalon lesions and death before 8 months of age, caused by a complex I deficiency; identified a missense mutation in the NDUFV1 gene; the mutation, p.Arg386His, affects a highly conserved residue
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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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Pathways associated with NDUFV1 elisa kitDiseases associated with NDUFV1 elisa kit
 Products by Pathway  Pathway Diagram
 Alzheimer's Disease Pathway antibodies  Alzheimer's Disease Pathway Diagram
 Alzheimer's Disease Pathway antibodies  Alzheimer's Disease Pathway Diagram
 Electron Transport Chain Pathway antibodies  Electron Transport Chain Pathway Diagram
 Huntington's Disease Pathway antibodies  Huntington's Disease Pathway Diagram
 Huntington's Disease Pathway antibodies  Huntington's Disease Pathway Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 NADH Dehydrogenase (ubiquinone) Fe-S Protein/flavoprotein Complex, Mitochondria Pathway antibodies  NADH Dehydrogenase (ubiquinone) Fe-S Protein/flavoprotein Complex, Mitochondria Pathway Diagram
 Oxidative Phosphorylation Pathway antibodies  Oxidative Phosphorylation Pathway Diagram
 Oxidative Phosphorylation Pathway antibodies  Oxidative Phosphorylation Pathway Diagram
 Oxidative Phosphorylation Pathway antibodies  Oxidative Phosphorylation Pathway Diagram
 Disease Name  Pubmed Publications
 Mitochondrial Diseases Antibodies  >13 publications with NDUFV1 and Mitochondrial Diseases
 Nervous System Diseases Antibodies  >12 publications with NDUFV1 and Nervous System Diseases
 Leigh Disease Antibodies  >6 publications with NDUFV1 and Leigh Disease
 Skin Diseases Antibodies  >3 publications with NDUFV1 and Skin Diseases
 Muscular Diseases Antibodies  >3 publications with NDUFV1 and Muscular Diseases
 MITOCHONDRIAL COMPLEX I DEFICIENCY Antibodies  >3 publications with NDUFV1 and MITOCHONDRIAL COMPLEX I DEFICIENCY
 Neuromuscular Diseases Antibodies  >3 publications with NDUFV1 and Neuromuscular Diseases
 Heart Diseases Antibodies  >2 publications with NDUFV1 and Heart Diseases
 Cardiovascular Diseases Antibodies  >2 publications with NDUFV1 and Cardiovascular Diseases
 Cardiomyopathies Antibodies  >2 publications with NDUFV1 and Cardiomyopathies
Organs/Tissues associated with NDUFV1 elisa kit
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >9 publications with NDUFV1 and Muscle
 Brain Antibodies  >6 publications with NDUFV1 and Brain
 Skin Antibodies  >5 publications with NDUFV1 and Skin
 Lymph Antibodies  >2 publications with NDUFV1 and Lymph
 Liver Antibodies  >2 publications with NDUFV1 and Liver
 Heart Antibodies  >2 publications with NDUFV1 and Heart
 Lymph Node Antibodies  >2 publications with NDUFV1 and Lymph Node
 Testis Antibodies  >1 publications with NDUFV1 and Testis
 Blood Antibodies  >1 publications with NDUFV1 and Blood
 Kidney Antibodies  >1 publications with NDUFV1 and Kidney
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