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Three genes are considered causative for ZLS: <jats:italic>KCNH1<\/jats:italic>, <jats:italic>KCNN3<\/jats:italic>, and <jats:italic>ATP6V1B2<\/jats:italic>. We report on a pair of female concordant monozygotic twins, both carrying a novel pathogenic variant in the <jats:italic>KCNN3<\/jats:italic> gene, identified using exome sequencing. Only six ZLS patients with the <jats:italic>KCNN3<\/jats:italic> pathogenic variant have been reported so far. The twins show facial dysmorphism, hypoplastic distal phalanges, aplasia or hypoplasia of nails, and hypertrichosis. During infancy, they showed mild developmental delays, mainly speech. They successfully completed secondary school education and are socio\u2010economically independent. Gingival overgrowth is absent in both individuals. Our patients exhibited an unusually mild phenotype compared to published cases, which is an important diagnostic finding for proper genetic counseling for Zimmermann\u2013Laband syndrome patients and their families.<\/jats:p>","DOI":"10.1002\/ajmg.a.62616","type":"journal-article","created":{"date-parts":[[2021,12,15]],"date-time":"2021-12-15T06:52:19Z","timestamp":1639551139000},"page":"1083-1087","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["<scp>Zimmermann\u2013Laband<\/scp> syndrome in monozygotic twins with a mild neurobehavioral phenotype lacking gingival overgrowth\u2014A case report of a novel <scp><i>KCNN3<\/i><\/scp> gene variant"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5585-8376","authenticated-orcid":false,"given":"Martin","family":"Schwarz","sequence":"first","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luk\u00e1\u0161","family":"Ryba","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"K\u0159epelov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronika","family":"Moslerov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Zelinov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marek","family":"Turnovec","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00falia","family":"Martinkov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Biology and Medical Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech 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Genetics, Second Faculty of Medicine Charles University in Prague and Motol University Hospital  Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,12,14]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-physiol-020911-153336"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2018.09.001"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2019.04.012"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2013.08.004"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M805700200"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41431-021-00818-9"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/bs.adgen.2020.03.002"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3282"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0030-4220(64)90506-7"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.4103\/ams.ams_203_17"},{"issue":"44","key":"e_1_2_11_12_1","first-page":"419","article-title":"Ueber 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