title: Concise Review: Cardiac Disease Modeling Using Induced Pluripotent Stem Cells.
abstract: Genetic cardiac diseases are major causes of morbidity and mortality. Although animal models have been created to provide some useful insights into the pathogenesis of genetic cardiac diseases, the significant species differences and the lack of genetic information for complex genetic diseases markedly attenuate the application values of such data. Generation of induced pluripotent stem cells (iPSCs) from patient-specific specimens and subsequent derivation of cardiomyocytes offer novel avenues to study the mechanisms underlying cardiac diseases, to identify new causative genes, and to provide insights into the disease aetiology. In recent years, the list of human iPSC-based models for genetic cardiac diseases has been expanding rapidly, although there are still remaining concerns on the level of functionality of iPSC-derived cardiomyocytes and their ability to be used for modeling complex cardiac diseases in adults. This review focuses on the development of cardiomyocyte induction from pluripotent stem cells, the recent progress in heart disease modeling using iPSC-derived cardiomyocytes, and the challenges associated with understanding complex genetic diseases. To address these issues, we examine the similarity between iPSC-derived cardiomyocytes and their ex vivo counterparts and how this relates to the method used to differentiate the pluripotent stem cells into a cardiomyocyte phenotype. We progress to examine categories of congenital cardiac abnormalities that are suitable for iPSC-based disease modeling.
authors.affiliation: Institute of Genetic Medicine, Newcastle University, The International Centre for Life, Central Parkway, Newcastle upon Tyne, United Kingdom.
authors.forename: Chunbo
authors.initials: C
authors.lastname: Yang
authors.affiliation: Princess Al Jawhara Center of Excellence in Research, King Abdulaziz University, Jeddah, Saudi Arabia.
authors.forename: Jumana
authors.initials: J
authors.lastname: Al-Aama
authors.affiliation: Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Kragujevac, Serbia.
authors.forename: Miodrag
authors.initials: M
authors.lastname: Stojkovic
authors.affiliation: Institute of Cardiovascular Sciences Core Technology, Manchester University, Manchester, United Kingdom.
authors.forename: Bernard
authors.initials: B
authors.lastname: Keavney
authors.affiliation: Institute of Cardiovascular Sciences Core Technology, Manchester University, Manchester, United Kingdom.
authors.forename: Andrew
authors.initials: A
authors.lastname: Trafford
authors.affiliation: Institute of Genetic Medicine, Newcastle University, The International Centre for Life, Central Parkway, Newcastle upon Tyne, United Kingdom.
authors.forename: Majlinda
authors.initials: M
authors.lastname: Lako
authors.affiliation: Institute of Genetic Medicine, Newcastle University, The International Centre for Life, Central Parkway, Newcastle upon Tyne, United Kingdom.
authors.forename: Lyle
authors.initials: L
authors.lastname: Armstrong
mesh_terms: D000818:Animals; D002454:Cell Differentiation / Q000502:physiology; D002478:Cells, Cultured; D006331:Heart Diseases / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000473:pathology* / Q000502:physiology; D032383:Myocytes, Cardiac / Q000473:pathology* / Q000502:physiology
keywords: Cardiac; Differentiation; Embryonic stem cells; Induced pluripotent stem cells