Article
All-trans retinoic acid and rapamycin normalize Hutchinson Gilford progeria fibroblast phenotype.
Oncotarget - 6 Oct 2015
Pellegrini Camilla, Columbaro Marta, Capanni Cristina, D'Apice Maria Rosaria, Cavallo Carola, Murdocca Michela, Lattanzi Giovanna, Squarzoni Stefano
Abstract excerpt
Hutchinson Gilford progeria syndrome is a fatal disorder characterized by accelerated aging, bone resorption and atherosclerosis, caused by a LMNA mutation which produces progerin, a mutant lamin A precursor. Progeria cells display progerin and prelamin A nuclear accumulation, altered histone methylation pattern, heterochromatin loss, increased DNA damage and cell cycle alterations. Since the LMNA promoter...
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