TY - JOUR AU - Rivera-Torres, Jose AU - Calvo, Conrado J. AU - Llach, Anna AU - Guzman-Martinez, Gabriela AU - Caballero, Ricardo AU - Gonzalez-Gomez, Cristina AU - Jimenez-Borreguero, Luis J. AU - Guadix, Juan A AU - Osorio, Fernando G AU - López-Otín, Carlos AU - Herraiz-Martínez, Adela AU - Cabello, Nuria AU - Vallmitjana, Alex AU - Benítez, Raul AU - Gordon, Leslie B AU - Jalife, Jose AU - Pérez-Pomares, José M AU - Tamargo, Juan AU - Delpón, Eva AU - Hove-Madsen, Leif AU - Filgueiras-Rama, David AU - Andres, Vicente PY - 2016 DO - 10.1073/pnas.1603754113 SN - 0027-8424 UR - http://hdl.handle.net/20.500.12105/7952 AB - Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease caused by defective prelamin A processing, leading to nuclear lamina alterations, severe cardiovascular pathology, and premature death. Prelamin A alterations also occur in... LA - eng PB - National Academy of Sciences KW - Hutchinson–Gilford progeria syndrome KW - calcium handling KW - connexin43 KW - prelamin A KW - progerin KW - Adolescent KW - Adult KW - Animals KW - Arrhythmias, Cardiac KW - Calcium KW - Cardiac Conduction System Disease KW - Child KW - Child, Preschool KW - Connexin 43 KW - Female KW - Heart KW - Humans KW - Male KW - Membrane Proteins KW - Metalloendopeptidases KW - Mice, Inbred C57BL KW - Mice, Knockout KW - Myocardium KW - Nuclear Lamina KW - Progeria KW - Sarcoplasmic Reticulum KW - Young Adult TI - Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations TY - journal article ER -