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

Stem cell research

PubMedID: 27345990

Fatima A, Ivanyuk D, Herms S, Heilmann-Heimbach S, O'Shea O, Chapman C, Izsvák Z, Farr M, Hescheler J, Šaric T. Generation of human induced pluripotent stem cell line from a patient with a long QT syndrome type 2. Stem Cell Res. 2016;16(2):304-7.
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).