Elevated levels of extracellular vesicles in progranulin-deficient mice and FTD-GRN Patients.

ABSTRACT:

Objective

The goal of this study was to investigate the effect of progranulin insufficiency on extracellular vesicles (EVs), a heterogeneous population of vesicles that may contribute to progression of neurodegenerative disease. Loss-of-function mutations in progranulin (GRN) are a major cause of frontotemporal dementia (FTD), and brains from GRN carriers with FTD (FTD-GRN) exhibit signs of lysosomal dysfunction. Lysosomal dysfunction may induce compensatory increases in secretion of exosomes, EVs secreted from the endolysosomal system, so we hypothesized that progranulin insufficiency would increase EV levels in the brain.

Methods

We analyzed levels and protein contents of brain EVs from Grn^-/- mice, which model the lysosomal abnormalities of FTD-GRN patients. We

SUBMITTER: Arrant AE 

PROVIDER: S-EPMC7732244 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature