title: A-type lamins and cardiovascular disease in premature aging syndromes.
abstract: Lamin A is a nuclear intermediate filament protein with important structural and regulatory roles in most differentiated mammalian cells. Excessive accumulation of its precursor prelamin A or the mutant form called 'progerin' causes premature aging syndromes. Progeroid 'laminopathies' are characterized by severe cardiovascular problems (cardiac electrical defects, vascular calcification and stiffening, atherosclerosis, myocardial infarction, and stroke) and premature death. Here, we review studies in cell and mouse models and patients that are unraveling how abnormal prelamin A and progerin accumulation accelerates cardiovascular disease and aging. This knowledge is essential for developing effective therapies to treat progeria and may help identify new mechanisms underlying normal aging.
authors.affiliation: Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), CIBER de Enfermedades Cardiovasculares, Madrid, Spain.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Dorado
authors.affiliation: Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), CIBER de Enfermedades Cardiovasculares, Madrid, Spain. Electronic address: vandres@cnic.es.
authors.forename: Vicente
authors.initials: V
authors.lastname: Andrés
mesh_terms: D000375:Aging / Q000378:metabolism / Q000473:pathology; D000818:Animals; D002318:Cardiovascular Diseases / Q000378:metabolism* / Q000473:pathology; D002467:Cell Nucleus / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D034904:Lamin Type A / Q000378:metabolism*; D011371:Progeria / Q000378:metabolism* / Q000473:pathology
chemical_list: D034904:Lamin Type A; C090764:prelamin A