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
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DOI - Digital Object Identifier
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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
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Result continuities
Project
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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
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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
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EID of the result in the Scopus database
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