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We previously reported that an equivalent point mutation severely compromised proton transport through the ATP synthase membrane domain (FO) in Saccharomyces cerevisiae and reduced the content of cytochrome c oxidase (Complex IV or COX) by 80%. Herein, we report that overexpression of the mitochondrial oxodicarboxylate carrier (Odc1p) considerably increases Complex IV abundance and tricarboxylic acid-mediated substrate-level phosphorylation of ADP coupled to conversion of \u03b1-ketoglutarate into succinate in m.8993T&amp;gt;G yeast. Consistently in m.8993T&amp;gt;G yeast cells, the retrograde signaling pathway was found to be strongly induced in order to preserve \u03b1-ketoglutarate production; when Odc1p was overexpressed, this stress pathway returned to an almost basal activity. Similar beneficial effects were induced by a partial uncoupling of the mitochondrial membrane with the proton ionophore, cyanide m-chlorophenyl hydrazone. This chemical considerably improved the glutamine-based, respiration-dependent growth of human cytoplasmic hybrid cells that are homoplasmic for the m.8993T&amp;gt;G mutation. These findings shed light on the interdependence between ATP synthase and Complex IV biogenesis, which could lay the groundwork for the creation of nutritional or metabolic interventions for attenuating the effects of mtDNA mutations.<\/jats:p>","DOI":"10.1093\/hmg\/ddz160","type":"journal-article","created":{"date-parts":[[2019,7,4]],"date-time":"2019-07-04T15:38:48Z","timestamp":1562254728000},"page":"3792-3804","source":"Crossref","is-referenced-by-count":21,"title":["Deregulating mitochondrial metabolite and ion transport has beneficial effects in yeast and human cellular models for NARP syndrome"],"prefix":"10.1093","volume":"28","author":[{"given":"Xin","family":"Su","sequence":"first","affiliation":[{"name":"Institut de Biochimie et G\u00e9n\u00e9tique Cellulaires, Universit\u00e9 Victor Segalen, 1 Rue Camille Saint-Sa\u00ebns, CEDEX 33077 Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malgorzata","family":"Rak","sequence":"additional","affiliation":[{"name":"Institut de Biochimie et G\u00e9n\u00e9tique Cellulaires, Universit\u00e9 Victor Segalen, 1 Rue Camille Saint-Sa\u00ebns, CEDEX 33077 Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Tetaud","sequence":"additional","affiliation":[{"name":"Institut de Biochimie et G\u00e9n\u00e9tique Cellulaires, Universit\u00e9 Victor Segalen, 1 Rue Camille Saint-Sa\u00ebns, CEDEX 33077 Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Godard","sequence":"additional","affiliation":[{"name":"Institut de Biochimie et G\u00e9n\u00e9tique Cellulaires, Universit\u00e9 Victor Segalen, 1 Rue Camille Saint-Sa\u00ebns, CEDEX 33077 Bordeaux, 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