Article
Neuronal VCP loss of function recapitulates FTLD-TDP pathology.
Cell reports - 20 Jul 2021
Wani Abubakar, Zhu Jiang, Ulrich Jason D, Eteleeb Abdallah, Sauerbeck Andrew D, Reitz Sydney J, Arhzaouy Khalid, Ikenaga Chiseko, Yuede Carla M, Pittman Sara K, Wang Feng, Li Shan, Benitez Bruno A, Cruchaga Carlos, Kummer Terrance T, Harari Oscar, Chou Tsui-Fen, Schröder Rolf, Clemen Christoph S, Weihl Conrad C
Abstract excerpt
The pathogenic mechanism by which dominant mutations in VCP cause multisystem proteinopathy (MSP), a rare neurodegenerative disease that presents as fronto-temporal lobar degeneration with TDP-43 inclusions (FTLD-TDP), remains unclear. To explore this, we inactivate VCP in murine postnatal forebrain neurons (VCP conditional knockout [cKO]). VCP cKO mice have cortical brain atrophy, neuronal loss,...
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