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September 10, 2025ASN NEUROOpen Access

Dysregulated Expression of Inflammasome and Extracellular Matrix Genes in C9orf72 -ALS/FTD Microglia

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Authors

LTLouise ThiryNPNisha S. PulimoodYTYe Man Tang

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Overview

RNA sequencing shows dysregulated inflammasome and extracellular matrix genes in C9orf72-HRE microglia, indicating a pro-inflammatory phenotype.

Key Points

  • Dysregulated expression of inflammasome genes leads to motor neuron death in ALS/FTD.
  • RNA sequencing shows altered extracellular matrix gene expression in C9orf72-HRE microglia.
  • Microglia from C9orf72-HRE iPSCs demonstrate a pro-inflammatory phenotype contributing to neuron degeneration.
  • C9orf72-HRE microglia interact with motor neurons, enhancing their death through secreted factors.

Cite This Study

Thiry et al. (2025) studied this question.

synapsesocial.com/papers/68c1bd2a54b1d3bfb60ee039https://doi.org/10.1080/17590914.2025.2542998
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