Dermal fibroblasts were donated by a 43 year old male patient with clinically diagnosed familial amyotrophic lateral sclerosis (ALS), carrying the SOD1^E101G 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 SOD1^E101G 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 SOD1 ^E101G mutation.

一名43岁男性患者经临床诊断为家族性肌萎缩性肌侧索硬化症（ALS），并携带SOD1 ^E101G突变，捐赠了皮肤成纤维细胞。使用多能性转录因子Oct4，Klf4，Sox2，c-Myc，Lin28和Nanog的重复mRNA转染，产生诱导性多能干细胞（iPSC）系UOWi007-A。iPSC携带SOD1 ^E101G基因型，具有正常的核型，表达预期的多能性标记，并且能够体外分化为内胚层，中胚层和外胚层谱系。该iPSC品系可能用于研究由SOD1 ^E101G突变引起的家族性ALS 。