Generation of ORIONi001-A induced pluripotent stem cell line for in vitro modeling of sporadic form of amyotrophic lateral sclerosis

We generated new in vitro model for sporadic form of amyotrophic lateral sclerosis by reprogramming isolated skin fibroblasts into iPSCs. Fibroblasts were reprogrammed with commercially available synthetic polycistronic, self-replicating RNA vector. As verified by FISH, an early passages of a new iPSC line showed mosaic karyotype (cells with normal and abnormal karyotype 46,XY,t(2;14)(q13;p12) were present), while late passages contained only cells with abnormal karyotype. New iPSCs differentiated into all three germ layers and formed a teratoma in nude mice. Our iPSC line represents a new model for therapy testing and drug development in the field of ALS research.

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Jan Strnadel, Romana Zahumenska, Vladimir Nosal, Marek Smolar, Juraj Marcinek, Michal Kalman, Stefan Juhas, Jana Juhasova, Hana Studenovska, Hugo Dumortier, Tomas Chromec, Henrieta Skovierova, Barbora Mitruskova, Ivan Kapralik, Sandra Mersakova, Dusan Brany, Erika Halasova

期刊 Stem Cell Res
影响因子 1.2
起止号 2020 Oct;48:101981.
种属 Human
技术平台抗体芯片
应用领域运动系统
研究方向运动系统
细胞类型干细胞
疾病类型肌萎缩症
检测蛋白 Oct-3/4,Nanog,SOX2,E-Cadherin,Alpha-Fetoprotein (AFP),GATA-4,HNF-3 beta/FoxA2,PDX-1/IPF1,SOX17,Otx2,TP63/TP73L,Goosecoid (Gsc),Snail,VEGF R2/KDR/Flk-1,HCG

Abstract

We generated new in vitro model for sporadic form of amyotrophic lateral sclerosis by reprogramming isolated skin fibroblasts into iPSCs. Fibroblasts were reprogrammed with commercially available synthetic polycistronic, self-replicating RNA vector. As verified by FISH, an early passages of a new iPSC line showed mosaic karyotype (cells with normal and abnormal karyotype 46,XY,t(2;14)(q13;p12) were present), while late passages contained only cells with abnormal karyotype. New iPSCs differentiated into all three germ layers and formed a teratoma in nude mice. Our iPSC line represents a new model for therapy testing and drug development in the field of ALS research.

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Abstract