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. Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.

Authors Strnadel J, Zahumenska R, Nosal V, Smolar M, Marcinek J, Kalman M, Juhas S, Juhasova J, Studenovska H, Dumortier H, Chromec T, Skovierova H, Mitruskova B, Kapralik I, Mersakova S, Brany D, Halasova E
Journal Stem cell research
Publication Date 2020 Oct;48:101981
PubMed 32919353
DOI 10.1016/j.scr.2020.101981

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