Article
Modeling xeroderma pigmentosum associated neurological pathologies with patients-derived iPSCs.
Protein & cell - 1 Mar 2016
Fu Lina, Xu Xiuling, Ren Ruotong, Wu Jun, Zhang Weiqi, Yang Jiping, Ren Xiaoqing, Wang Si, Zhao Yang, Sun Liang, Yu Yang, Wang Zhaoxia, Yang Ze, Yuan Yun, Qiao Jie, Izpisua Belmonte Juan Carlos, Qu Jing, Liu Guang-Hui
Abstract excerpt
Xeroderma pigmentosum (XP) is a group of genetic disorders caused by mutations of XP-associated genes, resulting in impairment of DNA repair. XP patients frequently exhibit neurological degeneration, but the underlying mechanism is unknown, in part due to lack of proper disease models. Here, we generated patient-specific induced pluripotent stem cells (iPSCs) harboring mutations in five different XP genes...
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