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We present two sisters with intellectual disability and muscular hypotonia born to non-consanguineous parents. Cogan ocular motor apraxia was present in both sisters. Body measurements were in a normal range. The mother and both daughters showed hypoplastic nails of the fifth toes. A missense variant in <jats:italic>SOX11<\/jats:italic> [c.139\u2009G\u2009&gt;\u2009A; p.(Gly47Ser)] in both sisters and their mother was identified. Since 2014, variants in <jats:italic>SOX11<\/jats:italic> are known to cause mild CSS. Most described patients showed intellectual disability, especially concerning acquired language. All of them had hypoplastic nails of the fifth toes. It is of note, that some of these patients show Cogan ocular motor apraxia. The facial dysmorphic features seem not to be specific. We suggest that the combination of Cogan ocular motor apraxia, hypoplastic nails of fifth toes, and developmental delay give the important diagnostic clue for a variant in the <jats:italic>SOX11<\/jats:italic> gene (OMIM 615866, MR 27).<\/jats:p>","DOI":"10.1038\/s41431-021-00865-2","type":"journal-article","created":{"date-parts":[[2021,3,31]],"date-time":"2021-03-31T03:53:40Z","timestamp":1617162820000},"page":"126-132","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Maternal transmission of a mild Coffin\u2013Siris syndrome phenotype caused by a SOX11 missense variant"],"prefix":"10.1038","volume":"30","author":[{"given":"Britta","family":"Hanker","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriele","family":"Gillessen-Kaesbach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irina","family":"H\u00fcning","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hermann-Josef","family":"L\u00fcdecke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dagmar","family":"Wieczorek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,3,31]]},"reference":[{"key":"865_CR1","first-page":"433","volume":"119","author":"GS Coffin","year":"1970","unstructured":"Coffin GS, Siris E. 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