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Vascular disease modeling using induced pluripotent stem cells: Focus in Hutchinson-Gilford Progeria Syndrome.
- Source :
-
Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2016 May 06; Vol. 473 (3), pp. 710-8. Date of Electronic Publication: 2015 Oct 20. - Publication Year :
- 2016
-
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.<br /> (Copyright © 2015 Elsevier Inc. All rights reserved.)
- Subjects :
- Animals
Drug Design
Drug Evaluation, Preclinical
Glycogen Storage Disease Type II physiopathology
Humans
Lamin Type A genetics
Tissue Engineering methods
Williams Syndrome physiopathology
Aortic Valve Stenosis physiopathology
Induced Pluripotent Stem Cells cytology
Progeria physiopathology
Vascular Diseases metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1090-2104
- Volume :
- 473
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Biochemical and biophysical research communications
- Publication Type :
- Academic Journal
- Accession number :
- 26474704
- Full Text :
- https://doi.org/10.1016/j.bbrc.2015.10.014