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In several hermaphroditic plants for example, sex is determined by an interaction between mitochondrial cytoplasmic male sterility (CMS) genes and nuclear restorer genes. Specifically, CMS involves aberrant mitochondrial genes that prevent pollen development and specific nuclear genes that restore it, leading to a mixture of female (male\u2010sterile) and hermaphroditic individuals in the population (gynodioecy). Such a mitochondrial\u2010nuclear sex determination system is thought to be rare outside plants. Here, we present one possible case of CMS in animals. We hypothesize that the only exception to the strict maternal mtDNA inheritance in animals, the doubly uniparental inheritance (DUI) system in bivalves, might have originated as a mitochondrial\u2010nuclear sex\u2010determination system. We document and explore similarities that exist between DUI and CMS, and we propose various ways to test our hypothesis.<\/jats:p>","DOI":"10.1002\/bies.202100283","type":"journal-article","created":{"date-parts":[[2022,2,16]],"date-time":"2022-02-16T11:05:24Z","timestamp":1645009524000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Did doubly uniparental inheritance (DUI) of mtDNA originate as a cytoplasmic male sterility (CMS) system?"],"prefix":"10.1002","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8286-486X","authenticated-orcid":false,"given":"Sophie","family":"Breton","sequence":"first","affiliation":[{"name":"D\u00e9partement des sciences biologiques Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald T.","family":"Stewart","sequence":"additional","affiliation":[{"name":"Department of Biology Acadia University  Wolfville Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Br\u00e9maud","sequence":"additional","affiliation":[{"name":"D\u00e9partement des sciences biologiques Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin C.","family":"Havird","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology The University of Texas at Austin  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chase H.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology The University of Texas at Austin  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter R.","family":"Hoeh","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences Kent State University  Kent Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,2,16]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-94139-4"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/aobpla\/plv037"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1086\/283342"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.2307\/2398798"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1558-5646.2012.01714.x"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-03908-3_2"},{"key":"e_1_2_12_8_1","first-page":"1","volume-title":"Evolutionary developmental biology","author":"Fruchard C.","year":"2017"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2009.01.048"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.es.24.110193.002301"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2006.12.004"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tree.2006.09.013"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1105\/tpc.015966"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.2741\/4531"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2014.09.002"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1098\/rstb.2020.0159"},{"key":"e_1_2_12_17_1","first-page":"81","article-title":"Sexuality and other aspects of reproduction in Anodonta (Pelecypoda: Unionidae)","volume":"15","author":"Heard W. 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