mitochondrially encoded NADH dehydrogenase 6, ELISA Kit

Human NADH-ubiquinone oxidoreductase chain 6 (MT-ND6) ELISA kit; MTND6; NADH dehydrogenase subunit 6; mitochondrially encoded NADH dehydrogenase 6

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of human MT-ND6. No significant cross-reactivity or interference between human MT-ND6 and analogues was observed.

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

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.

Small volumes of MT-ND6 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.

MBS936598 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the mitochondrially encoded NADH dehydrogenase 6 (MT-ND6) 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 MT-ND6. The ELISA analytical biochemical technique of the MBS936598 kit is based on MT-ND6 antibody-MT-ND6 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MT-ND6 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MT-ND6. 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(%).

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for MT-ND6 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any MT-ND6 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MT-ND6 is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of MT-ND6 bound in the initial step. The color development is stopped and the intensity of the color is measured.

18,622 Da

mitochondrially encoded NADH dehydrogenase 6

NADH dehydrogenase, subunit 6 (complex I); NADH dehydrogenase subunit 6

NADH-ubiquinone oxidoreductase chain 6

MTND6; NADH6; ND6  [Similar Products]

NU6M_HUMAN

MT-ND6: 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 MT-ND6 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Defects in MT-ND6 are a cause of Leber hereditary optic neuropathy with dystonia (LDYT); also called familial dystonia with visual failure and striatal lucencies. LDYT is part of a spectrum of Leber hereditary optic neuropathy. It is characterized by the association of optic atrophy and central vision loss with dystonia. Defects in MT-ND6 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS). MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness. Defects in MT-ND6 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to *****-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 subunit 6 family.

Protein type: Membrane protein, multi-pass; EC 1.6.5.3; Oxidoreductase; Membrane protein, integral; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: -

Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Leber Optic Atrophy

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