Generation of human induced pluripotent stem cell line from a patient with a long QT syndrome type 2


We report here the generation of human iPS cell line UKKi009-A from dermal fibroblasts of a patient carrying heterozygous mutation c.3035-3045delTCCCTCGATGC, p.Leu1012Pro (fs*55) in KCNH2 gene leading to long QT syndrome type 2 (LQT2). We used the Sleeping Beauty transposon-based plasmids expressing OSKM along with microRNAs 307/367 to reprogram the fibroblasts. The iPS cells possess pluripotent stem cell characteristics and differentiate to cell lineages of all three germ layers. This cell line can serve as a source for in vitro modeling of LQT2. This cell line is distributed by the European Collection of Authenticated Cell Cultures (ECACC). Copyright © 2016 University of Texas at Austin Dell Medical School. Published by Elsevier B.V. All rights reserved.

Authors Fatima A, Ivanyuk D, Herms S, Heilmann-Heimbach S, O'Shea O, Chapman C, Izsvák Z, Farr M, Hescheler J, Šarić T
Journal Stem cell research
Publication Date 2016 Mar;16(2):304-7
PubMed 27345990
DOI 10.1016/j.scr.2015.12.039

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