Neuronal VCP loss of function recapitulates FTLD-TDP pathology
Abstract
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, autophago-lysosomal dysfunction, and TDP-43...
Paper Details
Title
Neuronal VCP loss of function recapitulates FTLD-TDP pathology
Published Date
Jul 1, 2021
Journal
Volume
36
Issue
3
Pages
109399 - 109399
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