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February 8, 2026Open Access

Ablation of progranulin augments microglial activation and accelerates prion progression

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Authors

BLBei LiYSYiyue ShiWHWenyu Hou

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Overview

Reveals that progranulin deficiency enhances microglial activation and accelerates prion disease progression, suggesting therapeutic implications.

Key Points

  • The study aims to understand how progranulin deficiency affects microglial activation and prion disease progression.
  • Employed intracerebral inoculation with RML6 prions in Grn mice and controls.
  • Conducted histological analyses for microglial activation and astrocyte response.
  • Performed RNA-seq analysis to assess gene expression changes in microglial states.
  • Grn mice exhibited accelerated disease progression compared to controls.
  • Histological analysis showed elevated microglial activation and exacerbated astrogliosis in Grn mice.
  • RNA-seq indicated a shift of microglia from homeostatic to pro-inflammatory states due to PGRN deficiency.

Cite This Study

Li et al. (2025) studied this question.

synapsesocial.com/papers/698827570fc35cd7a88460bbhttps://doi.org/10.5167/uzh-290932
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Ablation of progranulin augments microglial activation and accelerates prion progression2025
  2. 2Aging is associated with behavioral alterations, lysosomal dysfunction, region-specific inflammation, and glutamatergic synaptic impairments in a Grn knockout mouse model of frontotemporal dementia2026
  3. 3Investigating the role and regulation of GPNMB in progranulin-deficient macrophages2024 · 1 citations
  4. 4Regulation of Progranulin Expression in Human Microglia and Proteolysis of Progranulin by Matrix Metalloproteinase-12 (MMP-12)2012 · 143 citations
  5. 5Progranulin Is a Survival Factor for Axotomized Retinal Ganglion Cells in Adult Mice2026