"NADH:ubichinon oxidoredukt\u00E1za \u010Di komplex I mitochondri\u00E1ln\u00EDho d\u00FDchac\u00EDho \u0159et\u011Bzce je slo\u017Eit\u00FD enzym s \u017Eivotn\u011B d\u016Fle\u017Eitou rol\u00ED v energetick\u00E9m metabolismu. D\u0159\u00EDve jsme vyvinuli hl\u00EDstov\u00FD model komplexu I spojen\u00FD s mitochondri\u00E1ln\u00ED dysfunkc\u00ED, jej\u00ED\u017E rysy jsou charakteristick\u00E9 pro mitochondri\u00E1ln\u00ED onemocn\u011Bn\u00ED jako je ml\u00E9\u010Dn\u00E1 acid\u00F3za a sn\u00ED\u017Een\u00E9 d\u00FDch\u00E1n\u00ED. Exprimovali jsme gen NDI1 ze Saccharomyces cerevisiae, v druhu Caenorhabditis elegans s po\u0161kozen\u00FDm komplexem I."@cs . . "1767" . "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." . "1"^^ . "RIV/00216208:11320/07:00004673" . "Expression; Ndi1p; alternative; ubiquinone; oxidoreductase; increases; mitochondrial; membrane; potential; elegans; model; mitochondrial; disease"@en . . . "Exprese proteinu Ndi1, alternativn\u00ED NADH:ubichinon oxidoredukt\u00E1zy, zvy\u0161uje mitochondri\u00E1ln\u00ED membr\u00E1nov\u00FD potenci\u00E1l v C. elegans, modelov\u00E9ho organismu pro mitochondri\u00E1ln\u00ED onemocn\u011Bn\u00ED"@cs . . "Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease"@en . . "G\u00E1\u0161kov\u00E1, Dana" . "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."@en . . . "Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease"@en . "11320" . "NL - Nizozemsko" . . . . "Z(MSM0021620835)" . . "1157;1163" . "RIV/00216208:11320/07:00004673!RIV08-MSM-11320___" . "Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease" . "Biochimica et Biophysica Acta - Bioenergetics" . . . "9" . . . . . . "7"^^ . "Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease" . "0005-2728" . "[2A97BE825364]" . . "421417" . . . . . "4"^^ . . "Exprese proteinu Ndi1, alternativn\u00ED NADH:ubichinon oxidoredukt\u00E1zy, zvy\u0161uje mitochondri\u00E1ln\u00ED membr\u00E1nov\u00FD potenci\u00E1l v C. elegans, modelov\u00E9ho organismu pro mitochondri\u00E1ln\u00ED onemocn\u011Bn\u00ED"@cs .