Cited by 134Prion Diseases and Protein MisfoldingNeuroinflammation and Neurodegeneration MechanismsAlzheimer's disease research and treatments

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A neuroprotective role for microglia in prion diseases

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Zhu, Caihong, Herrmann, Uli S., Falsig, Jeppe, Abakumova, Irina, Nuvolone, Mario, Schwarz, Petra, Frauenknecht, Katrin, Rushing, Elisabeth J., Aguzzi, Adriano·2016·Journal of Experimental Medicine

DOI 10.1084/jem.20151000PMC PMC4886355

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Abstract

Microglial activation is a hallmark of most neurodegenerative disorders, and is particularly conspicuous in prion diseases. However, the role of microglia, which function as both primary immune effector cells and professional phagocytes in the central nervous system, remains contentious in the context of neurodegeneration. Here, we evaluated the effect of microglial depletion/deficiency on prion pathogenesis. We found that ganciclovir-mediated microglial ablation on tga20/CD11b-thymidine kinase of Herpes simplex virus (HSVTK) cerebellar organotypic cultured slices markedly aggravated prion-induced neurotoxicity. A similar deterioration of disease was recapitulated in in vivo microglial depletion in prion-infected tga20/CD11b-HSVTK mice. Additionally, deficiency of microglia in interleukin 34 knockout (IL34−/−) mice again resulted in significantly augmented proteinase K–resistant prion protein deposition and accelerated prion disease progression. These results provide unambiguous evidence for a general protective role of microglia in prion pathogenesis.

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Background
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CSF1R-Mediated Myeloid Cell Depletion Prolongs Lifespan But Aggravates Distinct Motor Symptoms in a Model of Multiple System Atrophy
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Prion pathogenesis is unaltered in a mouse strain with a permeable blood-brain barrier
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Self-citation
Microglia-specific NF-κB signaling is a critical regulator of prion-induced glial inflammation and neuronal loss
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Genetically engineered cellular models of prion propagation
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Acute Neurotoxicity Models of Prion Disease
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Self-citation
Microglia have limited influence on early prion pathogenesis, clearance, or replication
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Self-citation
Downregulation of STAT3 transcription factor reverses synaptotoxic phenotype of reactive astrocytes associated with prion diseases
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Innate Immune Mechanisms in the Central Nervous System
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The Role of Glial Cells in Neurobiology and Prion Neuropathology
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Cell-based therapy against prion diseases
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Authors: Zhu, C., Herrmann, U. S., Falsig, J., Abakumova, I., Nuvolone, M., Schwarz, P., Frauenknecht, K., Rushing, E. J., & Aguzzi, A.
Affiliations: University Hospital Zurich (CH)
Corresponding author: Zhu, Caihong
Aguzzi, Adriano
ORCID: Zhu, Caihong
Herrmann, Uli S.
Nuvolone, Mario
Schwarz, Petra
Frauenknecht, Katrin
Rushing, Elisabeth J.
Aguzzi, Adriano
Venue: Journal of Experimental Medicine
Publisher: Rockefeller University Press
Published: 2016-05-16
Volume: 213
Issue: 6
Pages: 1047-1059
ISSN: 0022-1007 (ISSN-L)
1540-9538
DOI: 10.1084/jem.20151000
PMC: PMC4886355
OpenAlex: W2406691442
Cited by: 134
References: 44
Citations by year: 2026: 4 · 2025: 10 · 2024: 9 · 2023: 7 · 2022: 17 · 2021: 11 · 2020: 12 · 2019: 17 · 2018: 17 · 2017: 25 · 2016: 5
FWCI: 11.08
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: The Journal of Experimental Medicine (publishedVersion)
Europe PMC (PubMed Central) (publishedVersion, cc-by-nc-sa)
PubMed Central (publishedVersion, cc-by-nc-sa)
PubMed (publishedVersion)
Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, cc-by-nc)
Zurich Open Repository and Archive (University of Zurich) (submittedVersion, cc-by-nc)
Zurich Open Repository and Archive (University of Zurich)
Funders: European Research Council (250356)
Swiss National Foundation
Novartis Research Foundation
Stavros Niarchos Foundation
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: microglia, prion disease, neuroprotection, microglial depletion, ganciclovir, IL-34, prion protein deposition, neurodegeneration
MeSH terms: Animals, Interleukins, Prions, Prion Diseases, Microglia, Mice, Knockout, Mice

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A neuroprotective role for microglia in prion diseases

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