Cardiovascular Progerin Suppression and Lamin A Restoration Rescue Hutchinson-Gilford Progeria Syndrome

• Published in: Circulation (New York, N.Y.) Vol. 144; no. 22; pp. 1777 - 1794
• Main Authors: Sánchez-López, Amanda, Espinós-Estévez, Carla, González-Gómez, Cristina, Gonzalo, Pilar, Andrés-Manzano, María J, Fanjul, Víctor, Riquelme-Borja, Raquel, Hamczyk, Magda R, Macías, Álvaro, Del Campo, Lara, Camafeita, Emilio, Vázquez, Jesús, Barkaway, Anna, Rolas, Loïc, Nourshargh, Sussan, Dorado, Beatriz, Benedicto, Ignacio, Andrés, Vicente
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 30.11.2021
• Subjects: Animals; Disease Models, Animal; Humans; Lamin Type A - genetics; Lamin Type A - metabolism; Mice; Mice, Transgenic; Muscle, Smooth, Vascular - metabolism; Myocytes, Cardiac - metabolism; Myocytes, Smooth Muscle - metabolism; Original s; Progeria - genetics; Progeria - metabolism; smooth muscle; Hutchinson-Gilford progeria syndrome; cell; cardiac myocyte

Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder characterized by premature aging and death mainly because of myocardial infarction, stroke, or heart failure. The disease is provoked by progerin, a variant of lamin A expressed in most differentiated cells. Patients look healthy at birt...