Reduction of microglial progranulin does not exacerbate pathology or behavioral deficits in neuronal progranulin-insufficient mice

AE Arrant, AJ Filiano, AR Patel, MQ Hoffmann… - Neurobiology of …, 2019 - Elsevier
Loss-of-function mutations in progranulin (GRN), most of which cause progranulin
haploinsufficiency, are a major autosomal dominant cause of frontotemporal dementia …

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] Selective depletion of microglial progranulin in mice is not sufficient to cause neuronal ceroid lipofuscinosis or neuroinflammation

TL Petkau, N Kosior, K de Asis, C Connolly… - Journal of …, 2017 - Springer
Background Progranulin deficiency due to heterozygous null mutations in the GRN gene are
a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous …

Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice

TL Petkau, J Blanco, BR Leavitt - Neurobiology of Disease, 2017 - Elsevier
Progranulin deficiency due to heterozygous null mutations in the GRN gene is a common
cause of familial frontotemporal lobar degeneration (FTLD), while homozygous loss-of …

Core neuropathological abnormalities in progranulin-deficient mice are penetrant on multiple genetic backgrounds

TL Petkau, A Hill, BR Leavitt - Neuroscience, 2016 - Elsevier
Loss-of-function mutations in the progranulin gene (GRN) are a common cause of familial
frontotemporal lobar degeneration (FTLD). A high degree of heterogeneity in the age-of …

[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 …

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 …

[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 …

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 …

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 …