Progranulin deficiency results in sex-dependent alterations in microglia in response to demyelination

T Zhang, T Feng, K Wu, J Guo, AL Nana, G Yang… - Acta …, 2023 - Springer
Heterozygous mutations in the granulin (GRN) gene, resulting in the haploinsufficiency of
the progranulin (PGRN) protein, is a leading cause of frontotemporal lobar degeneration …

Depletion of progranulin reduces GluN2B-containing NMDA receptor density, tau phosphorylation, and dendritic arborization in mouse primary cortical neurons

F Longhena, M Zaltieri, J Grigoletto, G Faustini… - … of Pharmacology and …, 2017 - ASPET
Loss-of-function mutations in the progranulin (PGRN) gene are a common cause of familial
frontotemporal lobar degeneration (FTLD). This age-related neurodegenerative disorder …

Effects of exercise on progranulin levels and gliosis in progranulin-insufficient mice

AE Arrant, AR Patel, ED Roberson - eneuro, 2015 - eneuro.org
Loss-of-function mutations in progranulin (GRN) are one of the most common genetic
causes of frontotemporal dementia (FTD), a progressive, fatal neurodegenerative disorder …

[HTML][HTML] Commentary: possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice

TJ Sargeant - Frontiers in Aging Neuroscience, 2016 - frontiersin.org
The recent paper by Tanaka and colleagues details the neuropathological consequences of
progranulin knock out in a mouse model. Loss of progranulin function by GRN mutation …

Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice

Y Tanaka, JK Chambers, T Matsuwaki… - Acta neuropathologica …, 2014 - Springer
Introduction It has been shown that progranulin (PGRN) deficiency causes age-related
neurodegenerative diseases such as frontotemporal lobar degeneration (FTLD) and …

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

AE Arrant, SE Davis, RM Vollmer… - Annals of Clinical …, 2020 - Wiley Online Library
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 …

Cerebrospinal Fluid Progranulin, but Not Serum Progranulin, Is Reduced in GRN-Negative Frontotemporal Dementia

C Wilke, F Gillardon, C Deuschle, MA Hobert… - Neurodegenerative …, 2017 - karger.com
Abstract Background and Objective: Reduced progranulin levels are a hallmark of
frontotemporal dementia (FTD) caused by loss-of-function (LoF) mutations in the progranulin …

Progranulin expression in the developing and adult murine brain

TL Petkau, SJ Neal, PC Orban… - Journal of …, 2010 - Wiley Online Library
Frontotemporal lobar degeneration (FTLD) is a neurodegenerative condition characterized
by focal degeneration of the frontal and temporal lobes of the brain. Autosomal dominantly …

Intracellular proteolysis of progranulin generates stable, lysosomal granulins that are haploinsufficient in patients with frontotemporal dementia caused by GRN …

CJ Holler, G Taylor, Q Deng, T Kukar - Eneuro, 2017 - eneuro.org
Homozygous or heterozygous mutations in the GRN gene, encoding progranulin (PGRN),
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …

Cellular ageing, increased mortality and FTLD‐TDP‐associated neuropathology in progranulin knockout mice

H Wils, G Kleinberger, S Pereson… - The Journal of …, 2012 - Wiley Online Library
Loss‐of‐function mutations in progranulin (GRN) are associated with frontotemporal lobar
degeneration with intraneuronal ubiquitinated protein accumulations composed primarily of …