Article
Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson-Gilford Progeria Syndrome.
Nature communications - 18 Nov 2019
Aguado Julio, Sola-Carvajal Agustin, Cancila Valeria, Revêchon Gwladys, Ong Peh Fern, Jones-Weinert Corey Winston, Wallén Arzt Emelie, Lattanzi Giovanna, Dreesen Oliver, Tripodo Claudio, Rossiello Francesca, Eriksson Maria, d'Adda di Fagagna Fabrizio
Abstract excerpt
Hutchinson-Gilford progeria syndrome (HGPS) is a genetic disorder characterized by premature aging features. Cells from HGPS patients express progerin, a truncated form of Lamin A, which perturbs cellular homeostasis leading to nuclear shape alterations, genome instability, heterochromatin loss, telomere dysfunction and premature entry into cellular senescence. Recently, we reported that telomere dysfunction...
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