Generation of three iPSC lines from fibroblasts of a patient with Aicardi Goutières Syndrome mutated in TREX1

Publication date: Available online 14 September 2019Source: Stem Cell ResearchAuthor(s): R.M. Ferraro, G. Lanzi, S. Masneri, C. Barisani, G. Piovani, G. Savio, M. Cattalini, J. Galli, C. Cereda, M. Muzi-Falconi, S. Orcesi, E. Fazzi, S. GilianiAbstractFibroblasts from a patient with Aicardi Goutières Syndrome (AGS) carrying a compound heterozygous mutation in TREX1, were reprogrammed into induced pluripotent stem cells (iPSCs) to establish isogenic clonal stem cell lines: UNIBSi006-A, UNIBSi006-B, and UNIBSi006-C. Cells were transduced using the episomal Sendai viral vectors, containing human OCT4, SOX2, c-MYC and KLF4 transcription factors. The transgene-free iPSC lines showed normal karyotype, expressed pluripotent markers and displayed in vitro differentiation potential toward cells of the three embryonic germ layers.
Source: Stem Cell Research - Category: Stem Cells Source Type: research