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Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11320%2F07%3A00004673" target="_blank" >RIV/00216208:11320/07:00004673 - isvavai.cz</a>

  • Result on the web

  • DOI - Digital Object Identifier

Alternative languages

  • Result language

    angličtina

  • Original language name

    Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease

  • Original language description

    The NADH:ubiquinone oxidoreductase or complex I of the mitochondrial respiratory chain is an intricate enzyme with a vital role in energy metabolism. We have previously developed a nematode model of complex I-associated mitochondrial dysfunction that features hallmark characteristics of mitochondrial disease, such as lactic acidosis and decreased respiration. We have expressed the Saccharomyces cerevisiae NDI1 gene in a strain of Caenorhabditis elegans with an impaired complex I. Expression of Ndi1p produces marked improvements in animal fitness and reproduction, increases respiration rates and restores mitochondrial membrane potential to wild type levels. Ndi1p functionally integrates into the nematode respiratory chain and mitigates the deleterious effects of a complex I deficit. However, we have also shown that Ndi1p cannot substitute for the absence of complex I. Nevertheless, the yeast Ndi1p should be considered as a candidate for gene therapy in human diseases involving complex I

  • Czech name

    Exprese proteinu Ndi1, alternativní NADH:ubichinon oxidoreduktázy, zvyšuje mitochondriální membránový potenciál v C. elegans, modelového organismu pro mitochondriální onemocnění

  • Czech description

    NADH:ubichinon oxidoreduktáza či komplex I mitochondriálního dýchacího řetězce je složitý enzym s životně důležitou rolí v energetickém metabolismu. Dříve jsme vyvinuli hlístový model komplexu I spojený s mitochondriální dysfunkcí, jejíž rysy jsou charakteristické pro mitochondriální onemocnění jako je mléčná acidóza a snížené dýchání. Exprimovali jsme gen NDI1 ze Saccharomyces cerevisiae, v druhu Caenorhabditis elegans s poškozeným komplexem I.

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    BO - Biophysics

  • OECD FORD branch

Result continuities

  • Project

  • Continuities

    Z - Vyzkumny zamer (s odkazem do CEZ)

Others

  • Publication year

    2007

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Biochimica et Biophysica Acta - Bioenergetics

  • ISSN

    0005-2728

  • e-ISSN

  • Volume of the periodical

    1767

  • Issue of the periodical within the volume

    9

  • Country of publishing house

    NL - THE KINGDOM OF THE NETHERLANDS

  • Number of pages

    7

  • Pages from-to

    1157-1163

  • UT code for WoS article

  • EID of the result in the Scopus database