{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T21:39:08Z","timestamp":1791581948527,"version":"4.3.4"},"reference-count":41,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2018,6,21]],"date-time":"2018-06-21T00:00:00Z","timestamp":1529539200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Mitocon - Italian Association of Mitochondrial Diseases","award":["Donation to Carla Fiorentini"],"award-info":[{"award-number":["Donation to Carla Fiorentini"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The Escherichia coli protein toxin cytotoxic necrotizing factor 1 (CNF1), which acts on the Rho GTPases that are key regulators of the actin cytoskeleton, is emerging as a potential therapeutic tool against certain neurological diseases characterized by cellular energy homeostasis impairment. In this brief communication, we show explorative results on the toxin\u2019s effect on fibroblasts derived from a patient affected by myoclonic epilepsy with ragged-red fibers (MERRF) that carries a mutation in the m.8344A&gt;G gene of mitochondrial DNA. We found that, in the patient\u2019s cells, besides rescuing the wild-type-like mitochondrial morphology, CNF1 administration is able to trigger a significant increase in cellular content of ATP and of the mitochondrial outer membrane marker Tom20. These results were accompanied by a profound F-actin reorganization in MERRF fibroblasts, which is a typical CNF1-induced effect on cell cytoskeleton. These results point at a possible role of the actin organization in preventing or limiting the cell damage due to mitochondrial impairment and at CNF1 treatment as a possible novel strategy against mitochondrial diseases still without cure.<\/jats:p>","DOI":"10.3390\/ijms19071825","type":"journal-article","created":{"date-parts":[[2018,6,22]],"date-time":"2018-06-22T02:46:21Z","timestamp":1529635581000},"page":"1825","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study"],"prefix":"10.3390","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3075-3367","authenticated-orcid":false,"given":"Alessia","family":"Fabbri","sequence":"first","affiliation":[{"name":"Italian Center for Global Health, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Travaglione","sequence":"additional","affiliation":[{"name":"Italian Center for Global Health, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zaira","family":"Maroccia","sequence":"additional","affiliation":[{"name":"Italian Center for Global Health, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Guidotti","sequence":"additional","affiliation":[{"name":"Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1816-548X","authenticated-orcid":false,"given":"Ciro Leonardo","family":"Pierri","sequence":"additional","affiliation":[{"name":"Department of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari, via Orabona, 4, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7616-7008","authenticated-orcid":false,"given":"Guido","family":"Primiano","sequence":"additional","affiliation":[{"name":"Unit\u00e0 di Neurofisiopatologia, Area Neuroscienze, Universit\u00e0 Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli, 8, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8478-2799","authenticated-orcid":false,"given":"Serenella","family":"Servidei","sequence":"additional","affiliation":[{"name":"Unit\u00e0 di Neurofisiopatologia, Area Neuroscienze, Universit\u00e0 Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli, 8, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Loizzo","sequence":"additional","affiliation":[{"name":"Italian Center for Global Health, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1707-6282","authenticated-orcid":false,"given":"Carla","family":"Fiorentini","sequence":"additional","affiliation":[{"name":"Italian Center for Global Health, Istituto Superiore di Sanit\u00e0, Viale Regina Elena, 299, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2018,6,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"16080","DOI":"10.1038\/nrdp.2016.80","article-title":"Mitochondrial Diseases","volume":"2","author":"Gorman","year":"2016","journal-title":"Nat. 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