Modeling Inherited Cardiac Disorders : A Cell Is Worth a Thousand Genes

• Sallam Karim

  Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine

• Kodo Kazuki

  Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine

• Wu Joseph C.

  Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine

書誌事項

タイトル別名

• Modeling Inherited Cardiac Disorders
• – A Cell Is Worth a Thousand Genes –

抄録

Advances in the understanding and treatment of cardiac disorders have been thwarted by the inability to study beating human cardiac cells in vitro. Induced pluripotent stem cells (iPSCs) bypass this hurdle by enabling the creation of patient-specific iPSC-derived cardiomyocytes (iPSC-CMs). These cells provide a unique platform to study cardiac diseases in vitro, especially hereditary cardiac conditions. To date, iPSC-CMs have been used to successfully model arrhythmic disorders, showing excellent recapitulation of cardiac channel function and electrophysiologic features of long QT syndrome types 1, 2, 3, and 8, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Similarly, iPSC-CM models of dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) have shown robust correlation of predicted morphologic, contractile, and electrical phenotypes. In addition, iPSC-CMs have shown some features of the respective phenotypes for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), LEOPARD syndrome, Pompe’s disease, and Friedriech’s ataxia. In this review, we examine the progress of utilizing iPSC-CMs as a model for cardiac conditions and analyze the potential for the platform in furthering the biology and treatment of cardiac disorders.  (Circ J 2014; 78: 784–794)<br>

収録刊行物

• Circulation Journal

  Circulation Journal 78 (4), 784-794, 2014

  一般社団法人 日本循環器学会

キーワード

• Channelopathy
• Dilated cardiomyopathy
• Drug discovery
• Human-induced pluripotent stem cells
• Hypertrophic cardiomyopathy

詳細情報

• CRID: 1390282680085676288
• NII論文ID: 130003391060
• NII書誌ID: AA11591968
• DOI: 10.1253/circj.cj-14-0182
• COI: 1:STN:280:DC%2BC2crktFGmtw%3D%3D
• ISSN: 13474820
• ISSN: 13469843
• NDL書誌ID: 025355938
• PubMed: 24632794
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I025355938
• Web Site: https://www.jstage.jst.go.jp/article/circj/78/4/78_CJ-14-0182/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2014270560
• 本文言語コード: en
• データソース種別:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可