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

Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria

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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

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Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

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Losing protein in the brain: The case of progranulin

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Progranulin axis and recent developments in frontotemporal lobar degeneration

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Progranulin in the hematopoietic compartment protects mice from atherosclerosis

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Interaction between PGRN gene and the early trauma on clinical characteristics in patients with obsessive-compulsive disorder

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Progranulin from different gliocytes in the nucleus accumbens exerts distinct roles in FTD- and neuroinflammation-induced depression-like behaviors

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Repurposing of pexidartinib for microglia depletion and renewal

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Molecular and behavioural abnormalities in the FUS‐tg mice mimic frontotemporal lobar degeneration: Effects of old and new anti‐inflammatory therapies

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Lysosomal Dysfunction and Other Pathomechanisms in FTLD: Evidence from Progranulin Genetics and Biology

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Immunity impacts cognitive deficits across neurological disorders

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Granulin Knock Out Zebrafish Lack Frontotemporal Lobar Degeneration and Neuronal Ceroid Lipofuscinosis Pathology

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Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS

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Pharmacological and physiological roles of adipokines and myokines in metabolic-related dementia

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Galectin‐3 is upregulated in frontotemporal dementia patients with subtype specificity

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Could astrocytes be the primary target of an offending agent causing the primary degenerative diseases of the human central nervous system? A hypothesis

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Defective Lysosomal Lipid Catabolism as a Common Pathogenic Mechanism for Dementia

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Molecular Pathways Bridging Frontotemporal Lobar Degeneration and Psychiatric Disorders

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Approaches to develop therapeutics to treat frontotemporal dementia

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Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia

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Progress in Frontotemporal Dementia Research

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Elevated levels of extracellular vesicles in progranulin‐deficient mice and FTD‐GRN Patients

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Review: Modelling the pathology and behaviour of frontotemporal dementia

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Reducing inflammation and rescuing FTD-related behavioral deficits in progranulin-deficient mice with α7 nicotinic acetylcholine receptor agonists

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Microglia regulate brain progranulin levels through the endocytosis/lysosomal pathway

Dong, T., Tejwani, L., Jung, Y., Kokubu, H., Luttik, K., Driessen, T. M., & Lim, J. (2021) · JCI Insight · doi:10.1172/jci.insight.136147 · Cited by 13

U-shaped relationship between ageing and risk-taking behaviour in a wild-type rodent

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Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom?

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C9ORF72 knockdown triggers FTD-like symptoms and cell pathology in mice

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Chemical and genetic rescue of in vivo progranulin-deficient lysosomal and autophagic defects.

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Possible involvement of the cerebellum in motor-function impairment in progranulin-deficient mice

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ALS/FTD-linked TBK1 deficiency in microglia induces an aged-like microglial signature and drives social recognition deficits in mice

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The Role of White Matter Dysfunction and Leukoencephalopathy/Leukodystrophy Genes in the Aetiology of Frontotemporal Dementias: Implications for Novel Approaches to Therapeutics

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Biochemical, Biomarker, and Behavioral Characterization of the GrnR493X Mouse Model of Frontotemporal Dementia

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Genetic and Molecular Aspects of Frontotemporal Lobar Degeneration

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Wild-type bone marrow transplant partially reverses neuroinflammation in progranulin-deficient mice

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Age-dependent emergence of neurophysiological and behavioral abnormalities in progranulin-deficient mice

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Extracellular Cleavage of Microglia-Derived Progranulin Promotes Diet-Induced Obesity

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Genetic and functional characters of GRN p.T487I mutation in Taiwanese patients with atypical parkinsonian disorders

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Progranulin Deficient Mice Develop Nephrogenic Diabetes Insipidus

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Microglial progranulin differently regulates hypothalamic lysosomal function in lean and obese conditions via cleavage-dependent mechanisms

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Autophagy-Lysosome Dysfunction in Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

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Frontotemporal Dementia Patient Neurons With Progranulin Deficiency Display Protein DyshomeostasisPreprint

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Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice

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Glimepiride Administered in Chow Reversibly Impairs Glucose Tolerance in Mice

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Genetics of Frontotemporal Dementia

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Enhanced legumain activity links progranulin deficiency to TDP-43 pathology in frontotemporal lobar degenerationPreprint

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Increased Fat Taste Preference in Progranulin-Deficient Mice

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Matrine From Sophora Flavescens Attenuates on Collagen-Induced Osteoarthritis by Modulating the Activity of miR-29B-3P/PGRN Axis

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Pathology and pathophysiology

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Analysis of the Biomarkers for Neurodegenerative Diseases in Aged Progranulin Deficient Mice

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Targeting Granulin Haploinsufficiency in Frontotemporal Dementia: From Genetic Mechanisms to Therapeutics

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Opposing role of phagocytic receptors MERTK and AXL in Progranulin deficient FTD

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Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach

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Animal models of dementia

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The Development of Drug Therapies for Frontotemporal Dementia Caused by Progranulin Mutations

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Methods to Investigate the Protection Against Neurodegenerative Disorders Provided by Progranulin Gene Transfer in the Brain

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Frontotemporal Dementia

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Genetically Engineered Mouse Models for Alzheimer Disease and Frontotemporal Dementia

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Progranulin deficiency does not exacerbate TDP-43 pathology in TDP-43 transgenic mouse models

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Pharmacologictherapy for FTD and related disorders

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Alzheimer’s Disease and Frontotemporal Lobar Degeneration: Mouse Models

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Alzheimer’s Disease and Frontotemporal Lobar Degeneration: Mouse Models

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DissociationofFrontotemporalDementia-RelatedDeficits andNeuroinflammationinProgranulinHaploinsufficient Mice

Young, A. H. (2013)

HISTOLOGICAL AND BEHAVIORAL CONSEQUENCES OF REPEATED MILD TRAUMATIC BRAIN INJURY IN MICE

Hall, B. (2016) · UKnowledge (University of Kentucky) · doi:10.13023/etd.2016.165

Treatment

Dickerson, B. C. (2015) · Cambridge University Press eBooks · doi:10.1017/cbo9781316091586.019

Pathophysiologyand animal models of frontotemporal dementia

Warmus, B. A., & Roberson, E. D. (2015) · Cambridge University Press eBooks · doi:10.1017/cbo9781316091586.018

Nemo-like kinase regulates Progranulin levels in the brain through the microglial endocytosis-lysosomal pathwayPreprint

Dong, T., Kokubu, H., Driessen, T., Tejwani, L., & Lim, J. (2018) · bioRxiv · doi:10.1101/358010

Progranulin and Inflammation/Neuroinflammation

Hosokawa, M. (2019) · Springer eBooks · doi:10.1007/978-981-13-6186-9_7

Generation and Phenotyping of Progranulin-Deficient Mice

Matsuwaki, T. (2019) · Springer eBooks · doi:10.1007/978-981-13-6186-9_9

Premature termination codon readthrough restores progranulin expression in preclinical models of frontotemporal dementia and neuronal ceroid lipofuscinosis

Frew, J. (2021) · cIRcle (University of British Columbia) · doi:10.14288/1.0396548

Preclinical Models

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The patient's own bone marrow-derived stromal cells

de Munter, H. (2021) · Research Publications (Maastricht University) · doi:10.26481/dis.20210409jm

Neurobiology of FTD

Chung, D. C., Stankowski, J. N., & Petrucelli, L. (2017) · Oxford University Press eBooks · doi:10.1093/med/9780190681425.003.0056

Lipid Dysregulation Unveil the Intricate Interplay of Lysosomal and Mitochondrial Changes in Frontotemporal Dementia with GRN HaploinsufficiencyPreprint

Ondaro, J., Zúñiga-Elizari, J. L., Zufiría, M., Garciandia-Arcelus, M., Zulaica, M., Lafarga, M., Riancho, J., Holt, I. J., de Munaín, A. L., Moreno, F., et al. (2024) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2024.01.22.576606

Social Cognitive Rehabilitation for Neurodegenerative Disorders

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Nicorandil treatment improves survival and spatial learning in aged granulin knockout mice

Niedowicz, D. M., Wang, W., Prajapati, P., Zhong, Y., Fister, S. X., Rogers, C. B., Sompol, P. M., Powell, D. K., Patel, I., Norris, C. M., et al. (2024) · Brain Pathology · doi:10.1111/bpa.13312 · Cited by 1

Additional file 3 of Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations

Huang, M., Modeste, E. S., Dammer, E., Merino, P., Taylor, G., Duong, D. M., Deng, Q., Holler, C. J., Gearing, M., Dickson, D. W., et al. (2020) · Figshare · doi:10.6084/m9.figshare.13064301

Additional file 2 of Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations

Huang, M., Modeste, E. S., Dammer, E., Merino, P., Taylor, G., Duong, D. M., Deng, Q., Holler, C. J., Gearing, M., Dickson, D. W., et al. (2020) · Figshare · doi:10.6084/m9.figshare.13064298

Modeling C9ORF72 loss-of-function : a knockdown mouse model

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Étude de la composante neuroinflammatoire dans les DFT/SLA

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Differential lysosomal burden in the excitatory and inhibitory neurons of the FTD-GRN brain.

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Progranulin haploinsufficiency remodels the cerebral microvasculature and neurovascular unitPreprint

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P3‐001: PROGRANULIN KNOCK‐OUT MICE SHOW SYMPTOMS REMINISCENT OF FRONTOTEMPORAL DEMENTIA

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O1‐05‐03: Neuronal effects of progranulin deficiency contribute to frontotemporal dementia independent of neuroinflammation

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Dissociation of Frontotemporal Dementia–Related Deficits and Neuroinflammation in Progranulin Haploinsufficient Mice

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P1‐153: Core features of human frontotemporal dementia recapitulated in progranulin knockout mice

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

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