Disease‐specific phenotypes in iPSC‐derived neural stem cells with POLG mutations

Disease‐specific phenotypes in iPSC‐derived neural stem cells with POLG mutations

Abstract Mutations in POLG disrupt mtDNA replication and cause devastating diseases often with neurological phenotypes. Defining disease mechanisms has been hampered by limited access to human tissues, particularly neurons. Using patient cells carrying POLG mutations, we generated iPSCs and then neu...

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Main Authors: Kristina Xiao Liang, Cecilie Katrin Kristiansen, Sepideh Mostafavi, Guro Helén Vatne, Gina Alien Zantingh, Atefeh Kianian, Charalampos Tzoulis, Lena Elise Høyland, Mathias Ziegler, Roberto Megias Perez, Jessica Furriol, Zhuoyuan Zhang, Novin Balafkan, Yu Hong, Richard Siller, Gareth John Sullivan, Laurence A Bindoff
Format: Article
Language:English
Published: Springer Nature 2020-08-01
Series:EMBO Molecular Medicine
Subjects:
mitochondria
mitophagy
neural stem cells
POLG
reactive oxygen species
Online Access:https://doi.org/10.15252/emmm.202012146
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https://doi.org/10.15252/emmm.202012146

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