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We investigated the conservation of function between humans and D. discoideum and showed that DdFXN can substitute the human version in the interaction and activation of the Fe-S assembly supercomplex. We edited the D. discoideum fxn locus and isolated a defective mutant, clone 8, which presents landmarks of frataxin deficiency, such as a decrease in Fe-S cluster-dependent enzymatic functions, growth rate reduction, and increased sensitivity to oxidative stress. In addition, the multicellular development is affected as well as growing on bacterial lawn. We also assessed the rescuing capacity of DdFXN-G122V, a version that mimics a human variant present in some FA patients. While the expression of DdFXN-G122V rescues growth and enzymatic activity defects, as DdFXN does, multicellular development defects were only partially rescued. The results of the study suggest that this new D. discoideum strain offers a wide range of possibilities to easily explore diverse FA FXN variants. This can facilitate the development of straightforward drug screenings to look for new therapeutic strategies.<\/jats:p>","DOI":"10.1042\/bcj20230244","type":"journal-article","created":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T08:55:40Z","timestamp":1695027340000},"page":"1533-1551","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["CRISPR\/Cas9-based edition of frataxin gene in <i>Dictyostelium discoideum<\/i>"],"prefix":"10.1042","volume":"480","author":[{"given":"Hernan G.","family":"Gentili","sequence":"first","affiliation":[{"name":"1Instituto de Biociencias, Biotecnolog\u00eda y Biolog\u00eda Traslacional (iB3), Departamento de Fisiolog\u00eda y Biolog\u00eda Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda Florencia","family":"Pignataro","sequence":"additional","affiliation":[{"name":"1Instituto de Biociencias, Biotecnolog\u00eda y Biolog\u00eda Traslacional (iB3), Departamento de Fisiolog\u00eda y Biolog\u00eda Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Justo","family":"Olmos","sequence":"additional","affiliation":[{"name":"1Instituto de Biociencias, Biotecnolog\u00eda y Biolog\u00eda Traslacional (iB3), Departamento de Fisiolog\u00eda y Biolog\u00eda Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda Florencia","family":"Pavan","sequence":"additional","affiliation":[{"name":"2Instituto de Qu\u00edmica F\u00edsica de los Materiales, Medio Ambiente y Energ\u00eda (INQUIMAE), CONICET, FCEN, UBA, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lorena Itat\u00ed","family":"Iba\u00f1ez","sequence":"additional","affiliation":[{"name":"2Instituto de Qu\u00edmica F\u00edsica de los Materiales, Medio Ambiente y Energ\u00eda (INQUIMAE), CONICET, FCEN, UBA, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Javier","family":"Santos","sequence":"additional","affiliation":[{"name":"1Instituto de Biociencias, Biotecnolog\u00eda y Biolog\u00eda Traslacional (iB3), Departamento de Fisiolog\u00eda y Biolog\u00eda Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"},{"name":"3Departamento de Qu\u00edmica Biol\u00f3gica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2388-9493","authenticated-orcid":false,"given":"Francisco","family":"Velazquez Duarte","sequence":"additional","affiliation":[{"name":"1Instituto de Biociencias, Biotecnolog\u00eda y Biolog\u00eda Traslacional (iB3), Departamento de Fisiolog\u00eda y Biolog\u00eda Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente G\u00fciraldes 2160, Ciudad Universitaria, C1428EGA Buenos Aires, Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","published-online":{"date-parts":[[2023,10,5]]},"reference":[{"volume-title":"Dictyostelium","year":"2001","key":"2023100510211440000_BCJ-2023-0244C1"},{"key":"2023100510211440000_BCJ-2023-0244C2","doi-asserted-by":"publisher","first-page":"R1008","DOI":"10.1016\/j.cub.2010.09.051","article-title":"Dictyostelium","volume":"20","year":"2010","journal-title":"Curr. 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