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 …

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 …

[HTML][HTML] Preclinical interventions in mouse models of frontotemporal dementia due to progranulin mutations

SN Kashyap, NR Boyle, ED Roberson - Neurotherapeutics, 2023 - Elsevier
Heterozygous loss-of-function mutations in progranulin (GRN) cause frontotemporal
dementia (FTD), a leading cause of early-onset dementia characterized clinically by …

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 …

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 haploinsufficiency causes biphasic social dominance abnormalities in the tube test

AE Arrant, AJ Filiano, BA Warmus… - Genes, Brain and …, 2016 - Wiley Online Library
Loss‐of‐function mutations in progranulin (GRN) are a major autosomal dominant cause of
frontotemporal dementia (FTD), a neurodegenerative disorder in which social behavior is …

[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 …

Progranulin gene therapy improves lysosomal dysfunction and microglial pathology associated with frontotemporal dementia and neuronal ceroid lipofuscinosis

AE Arrant, VC Onyilo, DE Unger… - Journal of …, 2018 - Soc Neuroscience
Loss-of-function mutations in progranulin, a lysosomal glycoprotein, cause
neurodegenerative disease. Progranulin haploinsufficiency causes frontotemporal dementia …

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 …

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 …