Cardiomyocytes Derived From Pluripotent Stem Cells Recapitulate Electrophysiological Characteristics of an Overlap Syndrome of Cardiac Sodium Channel Disease | Zendy

Richard P. Davis | Zendy; Simona Casini | Zendy; Cathelijne W. van den Berg | Zendy; Maaike Hoekstra | Zendy; Carol Ann Remme | Zendy; Cheryl Dambrot | Zendy; Daniela Salvatori | Zendy; Dorien Wardvan Oostwaard | Zendy; Arthur A.M. Wilde | Zendy; Connie R. Bezzina | Zendy; Arie O. Verkerk | Zendy; Christian Freund | Zendy; Christine L. Mummery | Zendy

Open Access

Cardiomyocytes Derived From Pluripotent Stem Cells Recapitulate Electrophysiological Characteristics of an Overlap Syndrome of Cardiac Sodium Channel Disease

Author(s) -

Richard P. Davis,

Simona Casini,

Cathelijne W. van den Berg,

Maaike Hoekstra,

Carol Ann Remme,

Cheryl Dambrot,

Daniela Salvatori,

Dorien Wardvan Oostwaard,

Arthur A.M. Wilde,

Connie R. Bezzina,

Arie O. Verkerk,

Christian Freund,

Christine L. Mummery

Publication year - 2012

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.111.066092

Subject(s) - induced pluripotent stem cell , embryonic stem cell , sodium channel , mutation , myocyte , microbiology and biotechnology , electrophysiology , patch clamp , mutant , stem cell , wild type , medicine , biology , genetics , chemistry , gene , sodium , organic chemistry

Pluripotent stem cells (PSCs) offer a new paradigm for modeling genetic cardiac diseases, but it is unclear whether mouse and human PSCs can truly model both gain- and loss-of-function genetic disorders affecting the Na(+) current (I(Na)) because of the immaturity of the PSC-derived cardiomyocytes. To address this issue, we generated multiple PSC lines containing a Na(+) channel mutation causing a cardiac Na(+) channel overlap syndrome.

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