Loss of TBK1 activity leads to TDP-43 proteinopathy through lysosomal dysfunction in human motor neurons
Jin Hao, Michael F. Wells, Gengle Niu, Irune Guerra San Juan, Francesco Limone, Atsushi Fukuda, Marcel F. Leyton-Jaimes, Brian J. Joseph, Menglu Qian, Daniel A. Mordes, Bogdan Budnik, Zhixun Dou, Kevin C. Eggan
This study shows that TBK1 deficiency in human motor neurons causes TDP-43 proteinopathy through lysosomal dysfunction, and restoring TBK1 rescues the pathology.
bioRxiv · Oct 11, 2021 · PreprintNeuroscience