Induced Pluripotent Stem Cell Models of Progranulin-Deficient Frontotemporal Dementia Uncover Specific Reversible Neuronal Defects

The pathogenic mechanisms of frontotemporal dementia (FTD) remain poorly understood. Here we generated multiple induced pluripotent stem cell lines from a control subject, a patient with sporadic FTD, and an FTD patient with a novel heterozygous GRN mutation (progranulin [PGRN] S116X). In neurons a...

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Bibliographic Details
Main Authors: Sandra Almeida, Zhijun Zhang, Giovanni Coppola, Wenjie Mao, Kensuke Futai, Anna Karydas, Michael D. Geschwind, M. Carmela Tartaglia, Fuying Gao, Davide Gianni, Miguel Sena-Esteves, Daniel H. Geschwind, Bruce L. Miller, Robert V. Farese, Jr., Fen-Biao Gao
Format: Article
Language:English
Published: Elsevier 2012-10-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124712002884