Article
Loss of Tmem106b exacerbates FTLD pathologies and causes motor deficits in progranulin-deficient mice.
EMBO reports - 5 Oct 2020
Zhou Xiaolai, Brooks Mieu, Jiang Peizhou, Koga Shunsuke, Zuberi Aamir R, Baker Matthew C, Parsons Tammee M, Castanedes-Casey Monica, Phillips Virginia, Librero Ariston L, Kurti Aishe, Fryer John D, Bu Guojun, Lutz Cathleen, Dickson Dennis W, Rademakers Rosa
Abstract excerpt
Progranulin (PGRN) and transmembrane protein 106B (TMEM106B) are important lysosomal proteins implicated in frontotemporal lobar degeneration (FTLD) and other neurodegenerative disorders. Loss-of-function mutations in progranulin (GRN) are a common cause of FTLD, while TMEM106B variants have been shown to act as disease modifiers in FTLD. Overexpression of TMEM106B leads to lysosomal dysfunction, while loss of...
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