Article
MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients' Cells.
Cells - 10 Feb 2022
Harhouri Karim, Cau Pierre, Casey Frank, Guedenon Koffi Mawuse, Doubaj Yassamine, Van Maldergem Lionel, Mejia-Baltodano Gerardo, Bartoli Catherine, De Sandre-Giovannoli Annachiara, Lévy Nicolas
Abstract excerpt
Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging. Most classical HGPS patients carry a de novo point mutation within exon 11 of the LMNA gene encoding A-type lamins. This mutation activates a cryptic splice site, leading to the production of a truncated prelamin A, called...
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