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000277989 1001_ $$00000-0001-9478-5432$$aSanz-Moreno, Adrián$$b0
000277989 245__ $$aLoss of Ten1 in mice induces telomere shortening and models human dyskeratosis congenita.
000277989 260__ $$aWashington, DC [u.a.]$$bAssoc.$$c2025
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000277989 520__ $$aTelomere length regulation is essential for genome stability as short telomeres can trigger cellular senescence and apoptosis constituting an integral aspect of biological aging. Telomere biology disorders (TBDs) such as dyskeratosis congenita (DC) are rare, inherited diseases with known mutations in at least 16 different genes encoding components of the telomere maintenance complexes. 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 Ten1 deficiency created by CRISPR-Cas9-mediated exon 3 deletion. Ten1 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.
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000277989 650_7 $$2NLM Chemicals$$aTumor Suppressor Protein p53
000277989 650_7 $$2NLM Chemicals$$aTelomere-Binding Proteins
000277989 650_2 $$2MeSH$$aAnimals
000277989 650_2 $$2MeSH$$aDyskeratosis Congenita: genetics
000277989 650_2 $$2MeSH$$aDyskeratosis Congenita: pathology
000277989 650_2 $$2MeSH$$aDyskeratosis Congenita: metabolism
000277989 650_2 $$2MeSH$$aMice
000277989 650_2 $$2MeSH$$aTelomere Shortening: genetics
000277989 650_2 $$2MeSH$$aDisease Models, Animal
000277989 650_2 $$2MeSH$$aMice, Knockout
000277989 650_2 $$2MeSH$$aHumans
000277989 650_2 $$2MeSH$$aApoptosis: genetics
000277989 650_2 $$2MeSH$$aTumor Suppressor Protein p53: metabolism
000277989 650_2 $$2MeSH$$aTelomere: genetics
000277989 650_2 $$2MeSH$$aTelomere: metabolism
000277989 650_2 $$2MeSH$$aTelomere-Binding Proteins: genetics
000277989 650_2 $$2MeSH$$aTelomere-Binding Proteins: deficiency
000277989 650_2 $$2MeSH$$aSignal Transduction
000277989 7001_ $$00000-0002-6890-4984$$aBecker, Lore$$b1
000277989 7001_ $$0P:(DE-2719)2810906$$aXie, Kan$$b2$$udzne
000277989 7001_ $$00000-0003-4705-3399$$ada Silva-Buttkus, Patricia$$b3
000277989 7001_ $$aDragano, Nathalia R V$$b4
000277989 7001_ $$aAguilar-Pimentel, Antonio$$b5
000277989 7001_ $$aAmarie, Oana V$$b6
000277989 7001_ $$00000-0003-0816-9305$$aCalzada-Wack, Julia$$b7
000277989 7001_ $$00000-0002-0839-2761$$aKraiger, Markus$$b8
000277989 7001_ $$00000-0003-2475-0810$$aLeuchtenberger, Stefanie$$b9
000277989 7001_ $$00000-0002-6430-2732$$aSeisenberger, Claudia$$b10
000277989 7001_ $$aMarschall, Susan$$b11
000277989 7001_ $$aRathkolb, Birgit$$b12
000277989 7001_ $$0P:(DE-2719)2812562$$aScifo, Enzo$$b13
000277989 7001_ $$0P:(DE-2719)9002571$$aLiu, Ting$$b14$$udzne
000277989 7001_ $$0P:(DE-2719)9002059$$aThanabalasingam, Anoja$$b15$$udzne
000277989 7001_ $$00000-0001-6653-7386$$aSanchez-Vazquez, Raul$$b16
000277989 7001_ $$aMartinez, Paula$$b17
000277989 7001_ $$aBlasco, Maria A$$b18
000277989 7001_ $$00000-0001-6006-0740$$aSavage, Sharon A$$b19
000277989 7001_ $$aFuchs, Helmut$$b20
000277989 7001_ $$0P:(DE-2719)2289209$$aEhninger, Dan$$b21
000277989 7001_ $$aGailus-Durner, Valérie$$b22
000277989 7001_ $$0P:(DE-2719)9000920$$ade Angelis, Martin Hrabê$$b23
000277989 773__ $$0PERI:(DE-600)2810933-8$$a10.1126/sciadv.adp8093$$gVol. 11, no. 15, p. eadp8093$$n15$$peadp8093$$tScience advances$$v11$$x2375-2548$$y2025
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