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            <subfield code="0">(orcid)0000-0002-3217-1424</subfield>
            <subfield code="a">López-Gallardo, E.</subfield>
            <subfield code="u">Universidad de Zaragoza</subfield>
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            <subfield code="a">Food derived respiratory complex I inhibitors modify the effect of Leber hereditary optic neuropathy mutations</subfield>
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            <subfield code="a">Mitochondrial DNA mutations in genes encoding respiratory complex I polypeptides can cause Leber hereditary optic neuropathy. Toxics affecting oxidative phosphorylation system can also cause mitochondrial optic neuropathy. Some complex I inhibitors found in edible plants might differentially interact with these pathologic mutations and modify their penetrance. To analyze this interaction, we have compared the effect of rotenone, capsaicin and rolliniastatin-1 on cybrids harboring the most frequent Leber hereditary optic neuropathy mutations and found that m.3460G > A mutation increases rotenone resistance but capsaicin and rolliniastatin-1 susceptibility. Thus, to explain the pathogenicity of mitochondrial diseases due to mitochondrial DNA mutations, their potential interactions with environment factors will have to be considered.</subfield>
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            <subfield code="b">18 / 93 = 0.194</subfield>
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            <subfield code="a">Emperador, S.</subfield>
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