Abnormal synaptic architecture in iPSC-derived neurons from a multi-generational family with genetic Creutzfeldt-Jakob disease
In this article, Mostoslavsky, Harris, and colleagues present a large library of induced pluripotent stem cells (iPSCs) obtained from members of a multi-generational, Israeli family who harbor the E200K mutation in the prion protein, which causes genetic Creutzfeldt-Jakob disease. They differentiated the iPSCs into cortical neurons, and, despite the absence of pathological PrP formation, they documented an abnormal postsynaptic site. This collection will enable the exploration of key questions in the pathogenesis of genetic prion disease.
In this article, Mostoslavsky, Harris, and colleagues present a large library of induced pluripotent stem cells (iPSCs) obtained from members of a multi-generational, Israeli family who harbor the E200K mutation in the prion protein, which causes genetic Creutzfeldt-Jakob disease. They differentiated the iPSCs into cortical neurons, and, despite the absence of pathological PrP formation, they documented an abnormal postsynaptic site. This collection will enable the exploration of key questions in the pathogenesis of genetic prion disease.
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