Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B

Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B

The truncated mutant form of the charged multivesicular body protein 2B (CHMP2B) is causative for frontotemporal dementia linked to chromosome 3 (FTD3). CHMP2B is a constituent of the endosomal sorting complex required for transport (ESCRT) and, when mutated, disrupts endosome-to-lysosome traffickin...

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Bibliographic Details
Main Authors: Yu Zhang, Benjamin Schmid, Nanett K. Nikolaisen, Mikkel A. Rasmussen, Blanca I. Aldana, Mikkel Agger, Kirstine Calloe, Tina C. Stummann, Hjalte M. Larsen, Troels T. Nielsen, Jinrong Huang, Fengping Xu, Xin Liu, Lars Bolund, Morten Meyer, Lasse K. Bak, Helle S. Waagepetersen, Yonglun Luo, Jørgen E. Nielsen, Bjørn Holst, Christian Clausen, Poul Hyttel, Kristine K. Freude
Format: Article
Language:English
Published: Elsevier 2017-03-01
Series:Stem Cell Reports
Subjects:
frontotemporal dementia linked to chromosome 3 (FTD3)
CHMP2B
iPSC-derived neuron
disease modeling
endosome
mitochondria
iron homeostasis
oxidative stress
neurodegeneration
Online Access:http://www.sciencedirect.com/science/article/pii/S2213671117300279

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http://www.sciencedirect.com/science/article/pii/S2213671117300279

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