The mRNA-based reprogramming of fibroblasts from a SOD1E101G familial amyotrophic lateral sclerosis patient to induced pluripotent stem cell line UOWi007

• DOI: 10.1016/j.scr.2020.101701
• Publication Date: 2020-01-16T16:48:33Z
• Authors: Rachelle Balez; Tracey Berg; Monique Bax; Sonia Sanz Muñoz; Mauricio C. Cabra...; Martin Engel; Dzung Do-Ha; Claire H. Stevens; Dominic Rowe; Shu Yang; Ian P. Blair; Lezanne Ooi
• Keywords: 0301 basic medicine; Kruppel-Like Factor 4; 03 medical and health sciences; Superoxide Dismutase-1; QH301-705.5; Amyotrophic Lateral Sclerosis; Humans; RNA, Messenger; Biology (General); Fibroblasts; Cell Line; 3. Good health

Dermal fibroblasts were donated by a 43 year old male patient with clinically diagnosed familial amyotrophic lateral sclerosis (ALS), carrying the SOD1E101G mutation. The induced pluripotent stem cell (iPSC) line UOWi007-A was generated using repeated mRNA transfections for pluripotency transcription factors Oct4, Klf4, Sox2, c-Myc, Lin28 and Nanog. The iPSCs carried the SOD1E101G genotype and had a normal karyotype, expressed expected pluripotency markers and were capable of in vitro differentiation into endodermal, mesodermal and ectodermal lineages. This iPSC line may be useful for investigating familial ALS resulting from a SOD1E101G mutation.