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).
Fatima, A., Ivanyuk, D., Herms, S., Heilmann-Heimbach, S., O’Shea, O., Chapman, C., … Šarić, T. (2016). Generation of human induced pluripotent stem cell line from a patient with a long QT syndrome type 2. Stem Cell Research, 16(2), 304–307. https://doi.org/10.1016/j.scr.2015.12.039