Article
Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations.
Nature communications - 25 May 2017
Zhou Xiaolai, Sun Lirong, Bracko Oliver, Choi Ji Whae, Jia Yan, Nana Alissa L, Brady Owen Adam, Hernandez Jean C Cruz, Nishimura Nozomi, Seeley William W, Hu Fenghua
Abstract excerpt
Haploinsufficiency of progranulin (PGRN) due to mutations in the granulin (GRN) gene causes frontotemporal lobar degeneration (FTLD), and complete loss of PGRN leads to a lysosomal storage disorder, neuronal ceroid lipofuscinosis (NCL). Accumulating evidence suggests that PGRN is essential for proper lysosomal function, but the precise mechanisms involved are not known. Here, we show that PGRN facilitates...
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