We have generated UQACi001-A, a new induced pluripotent stem cell (iPSC) line derived from skin fibroblasts of a male patient with the generalized severe epidermolysis bullosa simplex phenotype (EBS-gen sev) and carrying the keratin 14 (K14) Arg125Ser mutation. Primary fibroblasts were reprogrammed using non-integrating Sendai virus vectors. The iPSC line is with a typical morphology of pluripotent stem cells and displayed normal molecular karyotype. It expressed pluripotency markers, is capable of differentiating into three embryonic germ layers and is genetically identical to the originating parental fibroblasts. The established iPSC model provides a powerful tool for: 1) establishing an iPSC-derived skin autograft; 2) identifying the biological pathways altered by the Arg125Ser mutation; 3) innovative drug screening and genome editing.

Name: Bchetnia Mbarka
Affiliation: UQAC
Edition: 2020