[PDF][PDF] Functional genomic analyses identify pathways dysregulated by progranulin deficiency, implicating Wnt signaling

EY Rosen, EM Wexler, R Versano, G Coppola, F Gao… - Neuron, 2011 - cell.com
Progranulin (GRN) mutations cause frontotemporal dementia (FTD), but GRN's function in
the CNS remains largely unknown. To identify the pathways downstream of GRN, we used …

Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN …

M Huang, E Modeste, E Dammer, P Merino… - Acta neuropathologica …, 2020 - Springer
Heterozygous, loss-of-function mutations in the granulin gene (GRN) encoding progranulin
(PGRN) are a common cause of frontotemporal dementia (FTD). Homozygous GRN …

Astroglial toxicity promotes synaptic degeneration in the thalamocortical circuit in frontotemporal dementia with GRN mutations

E Marsan, D Velmeshev, A Ramsey… - The Journal of …, 2023 - Am Soc Clin Investig
Mutations in the human progranulin (GRN) gene are a leading cause of frontotemporal lobar
degeneration (FTLD). While previous studies implicate aberrant microglial activation as a …

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 …

Restoring neuronal progranulin reverses deficits in a mouse model of frontotemporal dementia

AE Arrant, AJ Filiano, DE Unger, AH Young… - Brain, 2017 - academic.oup.com
Loss-of-function mutations in progranulin (GRN), a secreted glycoprotein expressed by
neurons and microglia, are a common autosomal dominant cause of frontotemporal …

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 …

[HTML][HTML] Tweaking progranulin expression: therapeutic avenues and opportunities

J Terryn, CM Verfaillie, P Van Damme - Frontiers in Molecular …, 2021 - frontiersin.org
Frontotemporal dementia (FTD) is a neurodegenerative disease, leading to behavioral
changes and language difficulties. Heterozygous loss-of-function mutations in progranulin …

Variations in the progranulin gene affect global gene expression in frontotemporal lobar degeneration

AS Chen-Plotkin, F Geser, JB Plotkin… - Human molecular …, 2008 - academic.oup.com
Frontotemporal lobar degeneration is a fatal neurodegenerative disease that results in
progressive decline in behavior, executive function and sometimes language. Disease …

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 …