Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury

.

• Publication year

  2022

• Venue

  Journal of Clinical Investigation

• Publication date

  2022-01-04

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1172/JCI157161PMID 34981791PMCID 8718132
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Synaptic dysfunction in progranulin-deficient mice.

  2012cited by this paper
• Cellular ageing, increased mortality and FTLD‐TDP‐associated neuropathology in progranulin knockout mice

  2012cited by this paper
• Core features of frontotemporal dementia recapitulated in progranulin knockout mice.

  2012cited by this paper
• Rescue of Progranulin Deficiency Associated with Frontotemporal Lobar Degeneration by Alkalizing Reagents and Inhibition of Vacuolar ATPase

  2011cited by this paper
• Extracellular progranulin protects cortical neurons from toxic insults by activating survival signaling.

  2011cited by this paper
• The Growth Factor Progranulin Binds to TNF Receptors and Is Therapeutic Against Inflammatory Arthritis in Mice

  2011influential reference
• Progranulin expression in the developing and adult murine brain

  2010cited by this paper
• Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging.

  2010cited by this paper
• Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

  2010cited by this paper
• Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

  2010cited by this paper
• Behavioral deficits and progressive neuropathology in progranulin‐deficient mice: a mouse model of frontotemporal dementia

  2010cited by this paper
• Increased caspase activation and decreased TDP‐43 solubility in progranulin knockout cortical cultures

  2010cited by this paper
• Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury.

  2009cited by this paper
• Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members

  2009cited by this paper
• Progranulin (PGRN) expression in ALS: an immunohistochemical study.

  2009cited by this paper
• Progranulin expression correlates with dense‐core amyloid plaque burden in Alzheimer disease mouse models

  2009cited by this paper
• Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration.

  2006cited by this paper
• Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

  2006cited by this paper
• Regulation of progranulin expression in myeloid cells.

  2006cited by this paper
• Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

  2006cited by this paper
• Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

  2006influential reference
• Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

  2006influential reference
• Automated microscope system for determining factors that predict neuronal fate.

  2005cited by this paper
• Genetic ablation of tumor necrosis factor‐alpha (TNF‐α) and pharmacological inhibition of TNF‐synthesis attenuates MPTP toxicity in mouse striatum

  2004cited by this paper
• Progranulin is a mediator of the wound response

  2003cited by this paper
• Parkinson's Disease Mechanisms and Models

  2003influential reference
• Progranulin (PC-cell-derived growth factor/acrogranin) regulates invasion and cell survival.

  2002cited by this paper
• Conversion of proepithelin to epithelins: roles of SLPI and elastase in host defense and wound repair.

  2002cited by this paper
• Tumor necrosis factor alpha is toxic to embryonic mesencephalic dopamine neurons.

  2001cited by this paper
• Cellular Localization of Gene Expression for Progranulin

  2000cited by this paper
• Fgf8 signalling from the AER is essential for normal limb development

  2000cited by this paper
• Granulins: the structure and function of an emerging family of growth factors.

  1998cited by this paper
• Microglial reaction in MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) induced Parkinson's disease mice model.

  1996cited by this paper

• Selective neuronal restoration of progranulin does not prevent the frontotemporal dementia like-phenotype of progranulin knockout mice

  2026cites this paper
• PGRN Counteracts the Associations of Abnormal Tau Proteins with Neurodegeneration and Cognitive Decline in Non-demented Adults: A Longitudinal Study

  2025cites this paper
• A novel c.1468 G > A GRN mutation causes frontotemporal dementia in a Chinese Han family

  2025cites this paper
• Microglial progranulin differently regulates hypothalamic lysosomal function in lean and obese conditions via cleavage-dependent mechanisms

  2025cites this paper
• A progranulin variant causing childhood interstitial lung disease responsive to anti-TNF-α biologic therapy.

  2025cites this paper
• Neuronal ceroid lipofuscinosis 11 (CLN11) presenting with early-onset cone-rod dystrophy and learning difficulties

  2025cites this paper
• Delivering Progranulin to Astrocytic Lysosomes Promotes Growth of Co‐Cultured Neurons

  2025cites this paper
• PU.1-driven enrichment enables microglia profiling from frozen brain tissue using the high-throughput Smart-seq3xpress method

  2025cites this paper
• Relationships of PGRN with sTREM2 in AD continuum and non-AD pathophysiology and their reciprocal roles in modulating amyloid pathology: two population-based study

  2025cites this paper
• TFEB overexpression alleviates autophagy-lysosomal deficits caused by progranulin insufficiency

  2025cites this paper
• Dose-Ranging Effects of the Intracerebral Administration of Atsttrin in Experimental Model of Parkinson’s Disease Induced by 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) in Mice

  2024cites this paper
• Investigating the role and regulation of GPNMB in progranulin-deficient macrophages

  2024cites this paper
• Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

  2024cites this paper
• Progranulin and neuropathological features of Alzheimer’s disease: longitudinal study

  2024cites this paper
• Blood-Based Biomarkers in Alzheimer’s Disease: Advancing Non-Invasive Diagnostics and Prognostics

  2024cites this paper
• Biochemical, Biomarker, and Behavioral Characterization of the GrnR493X Mouse Model of Frontotemporal Dementia

  2024cites this paper
• Investigating the Role and Regulation of GPNMB in Progranulin-deficient Macrophages

  2024cites this paper
• Selective neuronal expression of progranulin is sufficient to provide neuroprotective and anti-inflammatory effects after traumatic brain injury

  2024cites this paper
• Progranulin enhances the engraftment of transplanted human iPS cell-derived cerebral neurons

  2024cites this paper
• Sortilin is associated with progranulin deficiency and autism‐like behaviors in valproic acid‐induced autism rats

  2024cites this paper
• ASO-mediated knockdown of GPNMB in mutant-GRN and Grn-deficient peripheral myeloid cells disrupts lysosomal function and immune responses

  2024cites this paper
• Serum Progranulin Levels as Markers of Mammary Tumors in Women

  2024cites this paper
• Epigenetic modulation through BET bromodomain inhibitors as a novel therapeutic strategy for progranulin-deficient frontotemporal dementia

  2024cites this paper
• Consolidation of metabolomic, proteomic, and GWAS data in connective model of schizophrenia

  2023cites this paper
• Biochemical, biomarker, and behavioral characterization of the GrnR493X mouse model of frontotemporal dementia

  2023cites this paper
• Complement-mediated synapse loss in Alzheimer's disease: mechanisms and involvement of risk factors.

  2023cites this paper
• Progranulin-deficient macrophages cause cardiotoxicity under hypoxic conditions.

  2023cites this paper
• Progranulin and GPNMB: interactions in endo-lysosome function and inflammation in neurodegenerative disease

  2023cites this paper
• Progranulinopathy: A diverse realm of disorders linked to progranulin imbalances.

  2023cites this paper
• Targeting Progranulin as an Immuno-Neurology Therapeutic Approach

  2023cites this paper
• Immunity impacts cognitive deficits across neurological disorders

  2023cites this paper
• SorCS2 binds progranulin to regulate motor neuron development.

  2023cites this paper
• Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach

  2023cites this paper
• Progranulin is an FMRP target that influences macroorchidism but not behaviour in a mouse model of Fragile X Syndrome

  2023cites this paper
• Implications of MMP-12 in the pathophysiology of ischaemic stroke

  2023cites this paper
• IRAK-M deficiency exacerbates dopaminergic neuronal damage in a mouse model of sub-acute Parkinson’s disease

  2023cites this paper
• Progranulin deficiency results in sex-dependent alterations in microglia in response to demyelination

  2023cites this paper
• Glia in FTLD-GRN: from supporting cast to leading role

  2023cites this paper
• Progranulin Oncogenic Network in Solid Tumors

  2023cites this paper
• Depletion and activation of microglia impact metabolic connectivity of the mouse brain

  2023cites this paper
• SorCS2 binds progranulin and regulates motor axon outgrowth

  2023cites this paper
• Frontotemporal Dementia Patient Neurons With Progranulin Deficiency Display Protein Dyshomeostasis

  2023cites this paper
• Astroglial toxicity promotes synaptic degeneration in the thalamocortical circuit in frontotemporal dementia with GRN mutations

  2023cites this paper
• Potential Value of Cerebrospinal Fluid Progranulin in the Identification of Postoperative Delirium in Geriatrics Patients Undergoing Knee Replacement: The Perioperative Neurocognitive Disorder and Biomarker LifestylE Study

  2022cites this paper
• Progranulin from different gliocytes in the nucleus accumbens exerts distinct roles in FTD- and neuroinflammation-induced depression-like behaviors

  2022cites this paper
• Progranulin loss results in sex-dependent dysregulation of the peripheral and central immune system

  2022cites this paper
• Progranulin released from microglial lysosomes reduces neuronal ferroptosis after cerebral ischemia in mice

  2022cites this paper
• Longitudinal Brain Atrophy Rates in Presymptomatic Carriers of Genetic Frontotemporal Dementia

  2022cites this paper
• CSF glial markers are elevated in a subset of patients with genetic frontotemporal dementia

  2022cites this paper
• Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis

  2022cites this paper
• Ganoderma lucidum Modulates Inflammatory Responses following 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine (MPTP) Administration in Mice

  2022cites this paper
• Predicting conversion from mild cognitive impairment to Alzheimer’s disease with multimodal latent factors

  2022cites this paper
• Progranulin Preserves Autophagy Flux and Mitochondrial Function in Rat Cortical Neurons Under High Glucose Stress

  2022cites this paper
• Neuroimmune dysfunction in frontotemporal dementia: Insights from progranulin and C9orf72 deficiency.

  2022cites this paper
• Conserved gene signatures shared among MAPT mutations reveal defects in calcium signaling

  2022cites this paper
• Progranulin deficiency promotes persistent neuroinflammation and causes regional pathology in the hippocampus following traumatic brain injury

  2022influential citation
• Defects of Nutrient Signaling and Autophagy in Neurodegeneration

  2022cites this paper
• Progranulin improves neural development via the PI3K/Akt/GSK-3β pathway in the cerebellum of a VPA-induced rat model of ASD

  2022cites this paper
• The microbiota restrains neurodegenerative microglia in a model of amyotrophic lateral sclerosis

  2022cites this paper
• Cerebrospinal Fluid EV Concentration and Size Are Altered in Alzheimer’s Disease and Dementia with Lewy Bodies

  2022cites this paper
• Progranulin Promotes Functional Recovery in Rats with Acute Spinal Cord Injury via Autophagy-Induced Anti-inflammatory Microglial Polarization

  2022cites this paper
• Neuroinflammation in aucher disease, neuronal ceroid lipofuscinosis, and commonalities with Parkinson's disease.

  2022cites this paper
• Lysosomal dysfunction in neurodegeneration: emerging concepts and methods.

  2022cites this paper
• Loss of TREM2 rescues hyperactivation of microglia, but not lysosomal deficits and neurotoxicity in models of progranulin deficiency

  2022cites this paper
• Multiple Molecular Pathways Are Influenced by Progranulin in a Neuronal Cell Model–A Parallel Omics Approach

  2022cites this paper
• Analysis of the Biomarkers for Neurodegenerative Diseases in Aged Progranulin Deficient Mice

  2022cites this paper
• Outcomes of Progranulin Gene Therapy in the Retina are Dependent on Time of Delivery

  2021influential citation
• Differentially Expressed Circular RNAs in Peripheral Blood Mononuclear Cells of Patients with Parkinson's Disease

  2021cites this paper
• Lysosomal Dysfunction and Other Pathomechanisms in FTLD: Evidence from Progranulin Genetics and Biology.

  2021cites this paper
• Autosomal Dominant Frontotemporal Lobar Degeneration in a Filipino Family with Progranulin Mutation

  2021cites this paper
• Strategies and Tools for Studying Microglial-Mediated Synapse Elimination and Refinement

  2021cites this paper
• Human progranulin-expressing mice as a novel tool for the development of progranulin-modulating therapeutics.

  2021cites this paper
• Tumor necrosis factor superfamily molecules are increased in behavioral variant frontotemporal dementia and correlate with cortical atrophy: An exploratory investigation.

  2021cites this paper
• CSF sTREM2 is elevated in a subset in GRN-related frontotemporal dementia.

  2021cites this paper
• Neuroinflammation modulates the association of PGRN with cerebral amyloid-β burden.

  2021cites this paper
• Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice

  2021cites this paper
• A multifaceted role of progranulin in regulating amyloid-beta dynamics and responses

  2021cites this paper
• Progranulin in neurodegenerative dementia

  2021influential citation
• CSF PGRN May be Associated With Postoperative Delirium After Knee Replacement in Elderly Patients: The PNDABLE Study

  2021cites this paper
• Elevated serum and urine levels of progranulin (PGRN) as a predictor of microglia activation in the early phase of traumatic brain injury: a further link with the development of neurodegenerative diseases.

  2021cites this paper
• CSF PGRN May be Associated With Postoperative Delirium After Knee Replacement in Elderly Patients: A Prospective Nested Case-Control Study in the PNDABLE Study

  2021cites this paper
• Systematic Review: Genetic, Neuroimaging, and Fluids Biomarkers for Frontotemporal Dementia Across Latin America Countries

  2021cites this paper
• Loss of TREM2 reduces hyperactivation of progranulin deficient microglia but not lysosomal pathology

  2021cites this paper
• Tweaking Progranulin Expression: Therapeutic Avenues and Opportunities

  2021cites this paper
• Delivering progranulin to neuronal lysosomes protects against excitotoxicity

  2021influential citation
• Outcomes of progranulin gene therapy in the retina are dependent on time and route of delivery

  2021cites this paper
• TDP-43 and Inflammation: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

  2021cites this paper
• Loss of Lysosomal Proteins Progranulin and Prosaposin Associated with Increased Neurofibrillary Tangle Development in Alzheimer Disease.

  2021cites this paper
• Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD

  2021cites this paper
• IPSC-derived GRN-mutant astrocytes delay excitatory electrical development of neurons in a non-cell autonomous manner

  2021cites this paper
• Progranulin deficiency results in reduced bis(monoacylglycero)phosphate (BMP) levels and gangliosidosis

  2021cites this paper
• Microglia regulate brain progranulin levels through the endocytosis/lysosomal pathway

  2021cites this paper
• Neuroinflammation in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia and the Interest of Induced Pluripotent Stem Cells to Study Immune Cells Interactions With Neurons

  2021cites this paper
• Microglia and astrocyte involvement in neurodegeneration and brain cancer

  2021cites this paper
• Pgrn Alleviates Cerebral Amyloid-β Burden and Cognitive Impairments via Mediating Neuroin ammation

  2021cites this paper
• PGRN alleviates cerebral amyloid-β burden and cognitive impairments via mediating neuroin ammation

  2021cites this paper
• Integrated multi-omics analysis reveals 1 common and distinct dysregulated 2 pathways for genetic subtypes of 3 Frontotemporal Dementia

  2021cites this paper
• Neurotoxic microglia promote TDP-43 proteinopathy in progranulin deficiency

  2020cites this paper
• Pgrn Alleviates Cerebral Amyloid-β Burden and Cognitive Impairments via Mediating Neuroin ammation

  2020cites this paper
• Reduced plasma progranulin levels are associated with the severity of Parkinson's disease.

  2020cites this paper