Loss of Ten1 in mice induces telomere shortening and models human dyskeratosis congenita.

Adrián Sanz-Moreno, Lore Becker, Kan Xie, Patricia da Silva-Buttkus, Nathalia R V Dragano, Antonio Aguilar-Pimentel, Oana V Amarie, Julia Calzada-Wack, Markus Kraiger, Stefanie Leuchtenberger, Claudia Seisenberger, Susan Marschall, Birgit Rathkolb, Enzo Scifo, Ting Liu, Anoja Thanabalasingam, Raul Sanchez-Vazquez, Paula Martinez, Maria A Blasco, Sharon A Savage, Helmut Fuchs, Dan Ehninger, Valérie Gailus-Durner, Martin Hrabê de Angelis

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health (GmbH), Ingolstädter Landstraße 1, 85764 Neuherberg, Germany. · pubmed

Paper

Abstract

Telomere 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

The following analysis is generated by AI and may contain errors. It is separate from the authors’ abstract and is not peer review.

Research summary

The paper claims that the loss of TEN1 in mice leads to telomere shortening and models human dyskeratosis congenita. This research is relevant as it investigates the mechanisms of telomere biology, which is directly linked to aging and age-related diseases.

Author of this paper? Send a correction.