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The precise role of TEN1, part of the CST complex (CTC1, STN1, and TEN1), and the consequences of its loss of function in vivo are not yet known. We investigated the first viable murine model of\n            <jats:italic>Ten1<\/jats:italic>\n            deficiency created by CRISPR-Cas9\u2013mediated exon 3 deletion.\n            <jats:italic>Ten1<\/jats:italic>\n            homozygous knockout mice present with telomere attrition, short life span, skin hyperpigmentation, aplastic anemia, and cerebellar hypoplasia. Molecular analyses revealed a reduction of proliferating cells, increased apoptosis, and stem cell depletion with activation of the p53\/p21 signaling pathway. Our data demonstrate that Ten1 deficiency causes telomere shortening and associates with accelerated aging.\n          <\/jats:p>","DOI":"10.1126\/sciadv.adp8093","type":"journal-article","created":{"date-parts":[[2025,4,11]],"date-time":"2025-04-11T17:59:46Z","timestamp":1744394386000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":5,"title":["Loss of Ten1 in mice induces telomere shortening and models human dyskeratosis congenita"],"prefix":"10.1126","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9478-5432","authenticated-orcid":true,"given":"Adri\u00e1n","family":"Sanz-Moreno","sequence":"first","affiliation":[{"name":"Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum M\u00fcnchen, German Research Center for Environmental Health (GmbH), Ingolst\u00e4dter Landstra\u00dfe 1, 85764 Neuherberg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6890-4984","authenticated-orcid":true,"given":"Lore","family":"Becker","sequence":"additional","affiliation":[{"name":"Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum M\u00fcnchen, German Research Center for Environmental Health (GmbH), Ingolst\u00e4dter Landstra\u00dfe 1, 85764 Neuherberg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-2277-4422","authenticated-orcid":true,"given":"Kan","family":"Xie","sequence":"additional","affiliation":[{"name":"Translational Biogerontology Lab, German Center for Neurodegenerative Diseases (DZNE), Venusberg-Campus 1\/99, 53127 Bonn, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4705-3399","authenticated-orcid":true,"given":"Patricia","family":"da Silva-Buttkus","sequence":"additional","affiliation":[{"name":"Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum M\u00fcnchen, German Research Center for Environmental Health (GmbH), Ingolst\u00e4dter Landstra\u00dfe 1, 85764 Neuherberg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalia R. 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