[HTML][HTML] Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice

J Frew, HB Nygaard - Acta Neuropathologica Communications, 2021 - Springer
Frontotemporal lobar degeneration (FTLD) causes a spectrum of clinical presentations of
frontotemporal dementia (FTD), including progressive changes in behavior, personality …

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 …

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 …

A novel loss-of-function GRN mutation p.(Tyr229*): clinical and Neuropathological features

L Kuuluvainen, M Pöyhönen… - Journal of …, 2017 - content.iospress.com
Mutations in the progranulin (GRN) gene represent about 5–10% of frontotemporal lobar
degeneration (FTLD). We describe a proband with a novel GRN mutation c. 687T> A …

[HTML][HTML] FTD-associated behavioural and transcriptomic abnormalities in 'humanized'progranulin-deficient mice: A novel model for progranulin-associated FTD

B Life, TL Petkau, GNF Cruz, EI Navarro-Delgado… - Neurobiology of …, 2023 - Elsevier
Frontotemporal dementia (FTD) is an early onset dementia characterized by neuropathology
and behavioural changes. A common genetic cause of FTD is haploinsufficiency of the gene …

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 …

The novel GRN g. 1159_1160delTG mutation is associated with behavioral variant frontotemporal dementia

A Calvi, SMG Cioffi, P Caffarra… - Journal of …, 2015 - content.iospress.com
Mutations in progranulin gene (GRN) are a common cause of autosomal dominant
frontotemporal lobar degeneration and are associated with a wide phenotypic …

Modifiers of GRN-associated frontotemporal lobar degeneration

E Wauters, S Van Mossevelde, J Van der Zee… - Trends in molecular …, 2017 - cell.com
Heterozygous loss-of-function (LOF) mutations in the human progranulin gene (GRN) cause
frontotemporal lobar degeneration (FTLD) by a mechanism of haploinsufficiency. Patients …

[HTML][HTML] FTD-PSP is an unusual clinical phenotype in a frontotemporal dementia patient with a novel progranulin mutation

B Deng, Z Zheng, J Zheng, W Yang, Y Huang… - Aging and …, 2021 - ncbi.nlm.nih.gov
Progranulin (GRN) mutations are a major cause of frontotemporal dementia (FTD); the
spectrum of clinical phenotypes of FTD is much more extensive than previously reported …

[HTML][HTML] Progranulin axis and recent developments in frontotemporal lobar degeneration

AM Nicholson, J Gass, L Petrucelli… - Alzheimer's Research & …, 2012 - Springer
Frontotemporal lobar degeneration (FTLD) is a devastating neurodegenerative disease that
is the second most common form of dementia affecting individuals under age 65. The most …