Article
Restoration of progranulin expression rescues cortical neuron generation in an induced pluripotent stem cell model of frontotemporal dementia.
Stem cell reports - 13 Jan 2015
Raitano Susanna, Ordovàs Laura, De Muynck Louis, Guo Wenting, Espuny-Camacho Ira, Geraerts Martine, Khurana Satish, Vanuytsel Kim, Tóth Balazs I, Voets Thomas, Vandenberghe Rik, Cathomen Toni, Van Den Bosch Ludo, Vanderhaeghen Pierre, Van Damme Philip, Verfaillie Catherine M
Abstract excerpt
To understand how haploinsufficiency of progranulin (PGRN) causes frontotemporal dementia (FTD), we created induced pluripotent stem cells (iPSCs) from patients carrying the GRN(IVS1+5G > C) mutation (FTD-iPSCs). FTD-iPSCs were fated to cortical neurons, the cells most affected in FTD. Although g...
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