Microglial NFκB-TNFα hyperactivation induces obsessive–compulsive behavior in mouse models of progranulin-deficient frontotemporal dementia

G Krabbe, SS Minami, JI Etchegaray… - Proceedings of the …, 2017 - National Acad Sciences
Frontotemporal dementia (FTD) is the second most common dementia before 65 years of
age. Haploinsufficiency in the progranulin (GRN) gene accounts for 10% of all cases of …

Dissociation of frontotemporal dementia–related deficits and neuroinflammation in progranulin haploinsufficient mice

AJ Filiano, LH Martens, AH Young… - Journal of …, 2013 - Soc Neuroscience
Frontotemporal dementia (FTD) is a neurodegenerative disease with hallmark deficits in
social and emotional function. Heterozygous loss-of-function mutations in GRN, the …

Core features of frontotemporal dementia recapitulated in progranulin knockout mice

N Ghoshal, JT Dearborn, DF Wozniak, NJ Cairns - Neurobiology of disease, 2012 - Elsevier
Frontotemporal dementia (FTD) is typified by behavioral and cognitive changes manifested
as altered social comportment and impaired memory performance. To investigate the …

Progranulin deficiency induces overactivation of WNT5A expression via TNF-α/NF-κB pathway in peripheral cells from frontotemporal dementia-linked granulin …

C Alquézar, A de la Encarnación, F Moreno… - Journal of Psychiatry and …, 2016 - jpn.ca
Background: Loss-of-function progranulin gene (GRN) mutations have been identified as the
major cause of frontotemporal lobar degeneration with transactive response (TAR) DNA …

Inflammatory molecules in frontotemporal dementia: cerebrospinal fluid signature of progranulin mutation carriers

D Galimberti, R Bonsi, C Fenoglio, M Serpente… - Brain, behavior, and …, 2015 - Elsevier
Mutations in progranulin gene (GRN) are one of the major causes of autosomal dominant
Frontotemporal Lobar Degeneration (FTLD). Progranulin displays anti-inflammatory …

Synaptic dysfunction in progranulin-deficient mice

TL Petkau, SJ Neal, A Milnerwood, A Mew, AM Hill… - Neurobiology of …, 2012 - Elsevier
Progranulin haploinsufficiency is a common cause of familial frontotemporal dementia
(FTD), but the role of progranulin in the brain is poorly understood. To investigate the role of …

Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD

Y Wu, W Shao, TW Todd, J Tong, M Yue, S Koga… - Cell reports, 2021 - cell.com
Loss-of-function mutations in the progranulin gene (GRN), which encodes progranulin
(PGRN), are a major cause of frontotemporal dementia (FTD). GRN-associated FTD is …

[HTML][HTML] Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

F Yin, M Dumont, R Banerjee, Y Ma, H Li… - The FASEB …, 2010 - ncbi.nlm.nih.gov
Progranulin haploinsufficiency causes frontotemporal dementia with tau-negative, ubiquitin-
positive neuronal inclusion pathology. In this study, we showed that progranulin-deficient …

Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay

AD Nguyen, TA Nguyen, J Zhang… - Proceedings of the …, 2018 - National Acad Sciences
Frontotemporal dementia (FTD) is the most common neurodegenerative disorder in
individuals under age 60 and has no treatment or cure. Because many cases of FTD result …

Progranulin deficiency causes impairment of autophagy and TDP-43 accumulation

MC Chang, K Srinivasan, BA Friedman… - Journal of Experimental …, 2017 - rupress.org
Loss-of-function mutations in GRN cause frontotemporal dementia (FTD) with transactive
response DNA-binding protein of 43 kD (TDP-43)–positive inclusions and neuronal ceroid …