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From patient-specific induced pluripotent stem cells to clinical translation in long QT syndrome Type 2

15 Jan 2019

Abstract excerpt

AIMS: Having shown that Lumacaftor rescued the hERG trafficking defect in the induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) of two LQT2 patients, we tested whether the commercial association Lumacaftor + Ivacaftor (LUM + IVA) could shorten the QTc in the same two patients. METHODS AND RESULTS: After hospital admission and 1 day of baseline recordings, half dose LUM + IVA was administered on Day...

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PMID: 30753398
DOI: 10.1093/eurheartj/ehz023

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