title: Vascular disease modeling using induced pluripotent stem cells: Focus in Hutchinson-Gilford Progeria Syndrome.
abstract: Induced pluripotent stem cells (iPSCs) represent today an invaluable tool to create disease cell models for modeling and drug screening. Several lines of iPSCs have been generated in the last 7 years that changed the paradigm for studying diseases and the discovery of new drugs to treat them. In this article we focus our attention to vascular diseases in particular Hutchinson-Gilford Progeria Syndrome (HGPS), a devastating premature aging disease caused by a mutation in the lamin A gene. In general, patients die because of myocardial infarction or stroke. Because the patients are fragile the isolation of a particular type of cells is very difficult. Therefore in the last 5 years, researchers have used cells derived from iPSCs to model aspects of the HGPS and to screen libraries of chemicals to retard or treat the disease.
authors.affiliation: CNC-Center for Neurosciences and Cell Biology, University of Coimbra, Coimbra, Portugal; Centre of Biological Engineering, University of Minho, Campus de Gualtar, Braga, Portugal.
authors.forename: P R
authors.initials: PR
authors.lastname: Pitrez
authors.affiliation: CNC-Center for Neurosciences and Cell Biology, University of Coimbra, Coimbra, Portugal.
authors.forename: S C
authors.initials: SC
authors.lastname: Rosa
authors.affiliation: CNC-Center for Neurosciences and Cell Biology, University of Coimbra, Coimbra, Portugal; Faculté des Sciences Jean Perrin, Université d'Artois, Lens, France; Doctoral Programme in Experimental Biology and Biomedicine (PDBEB), University of Coimbra, Coimbra, Portugal; Institute for Interdisciplinary Research (IIIUC), University of Coimbra, Coimbra, Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Praça
authors.affiliation: CNC-Center for Neurosciences and Cell Biology, University of Coimbra, Coimbra, Portugal; Institute for Interdisciplinary Research (IIIUC), University of Coimbra, Coimbra, Portugal. Electronic address: lino@biocant.pt.
authors.forename: L
authors.initials: L
authors.lastname: Ferreira
mesh_terms: D000818:Animals; D001024:Aortic Valve Stenosis / Q000503:physiopathology*; D015195:Drug Design; D004353:Drug Evaluation, Preclinical; D006009:Glycogen Storage Disease Type II / Q000503:physiopathology; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000166:cytology*; D034904:Lamin Type A / Q000235:genetics; D011371:Progeria / Q000503:physiopathology*; D023822:Tissue Engineering / Q000379:methods; D014652:Vascular Diseases / Q000378:metabolism*; D018980:Williams Syndrome / Q000503:physiopathology
chemical_list: D034904:Lamin Type A
keywords: Drug modeling; Drug screening; Induced pluripotent stem cells; Progeria disease; Vascular cells