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These diseases arise from mutations in either mitochondrial DNA or nuclear DNA. Among nuclear DNA-related cases, mutations in\n                    <jats:italic>POLG<\/jats:italic>\n                    and\n                    <jats:italic>POLG2<\/jats:italic>\n                    , which encode subunits of mitochondrial DNA polymerase \u03b3, are particularly significant, causing conditions such as Alpers\u2013Huttenlocher syndrome and progressive external ophthalmoplegia. Model organisms have been instrumental in elucidating POLG-related disease mechanisms and advancing therapeutic strategies.\n                    <jats:italic>Saccharomyces cerevisiae<\/jats:italic>\n                    (budding yeast) provided insights into fundamental mitochondrial functions, while\n                    <jats:italic>Caenorhabditis elegans<\/jats:italic>\n                    (roundworm) helped explore POLG\u2019s roles in multicellular organisms.\n                    <jats:italic>Drosophila melanogaster<\/jats:italic>\n                    (fruit fly) has been pivotal in studying neurological aspects, and\n                    <jats:italic>Mus musculus<\/jats:italic>\n                    (mouse) models contributed to understanding systemic effects in mammals. Recently,\n                    <jats:italic>Danio rerio<\/jats:italic>\n                    (zebrafish) has emerged as a promising vertebrate model for drug screening, due to its optical transparency and genetic tractability. Each model system offers unique advantages, collectively bridging the gap between basic research and clinical applications. This review will examine in vivo models used in POLG disorder research, highlighting their contributions to understanding disease mechanisms and therapeutic advancements.\n                  <\/jats:p>","DOI":"10.1038\/s41419-025-08366-6","type":"journal-article","created":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T18:44:33Z","timestamp":1766774673000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Model organisms in POLG-related disorders: insights from yeast to multicellular systems"],"prefix":"10.1038","volume":"17","author":[{"given":"Raquel","family":"Bra\u00f1as Casas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0651-7409","authenticated-orcid":false,"given":"Giovanni","family":"Risato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9601-3567","authenticated-orcid":false,"given":"Alessandro","family":"Zuppardo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6050-0566","authenticated-orcid":false,"given":"Carlo","family":"Viscomi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0803-8236","authenticated-orcid":false,"given":"Francesco","family":"Argenton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6771-5233","authenticated-orcid":false,"given":"Mara","family":"Doimo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Facchinello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5444-9853","authenticated-orcid":false,"given":"Natascia","family":"Tiso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,12,26]]},"reference":[{"issue":"10","key":"8366_CR1","doi-asserted-by":"publisher","first-page":"1150","DOI":"10.1089\/ars.2011.4085","volume":"16","author":"VG Antico Arciuch","year":"2012","unstructured":"Antico Arciuch VG, Elguero ME, Poderoso JJ, Carreras MC. 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