- Generation of three human iPSC lines from PLAN (PLA2G6-associated neurodegeneration) patients.
Generation of three human iPSC lines from PLAN (PLA2G6-associated neurodegeneration) patients.
Stem cell research (2021-06-06)
Candela Machuca, Marta Correa-Vela, Deyanira García-Navas, Alejandra Darling, Irene Villalón-García, José Antonio Sánchez-Alcázar, Belén Pérez-Dueñas, Slaven Erceg, Carmen Espinós
PMID34087982
摘要
The human iPSC cell lines, PLANFiPS1-Sv4F-1 (RCPFi004-A), PLANFiPS2-Sv4F-1 (RCPFi005-A), PLANFiPS3-Sv4F-1 RCPFi006-A), derived from dermal fibroblast from three patients suffering PLAN (PLA2G6-associated neurodegeneration; MIM 256600) caused by mutations in the PLA2G6 gene, was generated by non-integrative reprogramming technology using OCT3/4, SOX2, CMYC and KLF4 reprogramming factors. The pluripotency was assessed by immunocytochemistry and RT-PCR. Differentiation capacity was verified in vitro. This iPSC line can be further differentiated toward affected cells to better understand molecular mechanisms of disease and pathophysiology.
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