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Related Experiment Video

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Innate immune activation and aberrant function in the R6/2 mouse model and Huntington's disease iPSC-derived

Julien Gasser1, Gaelle Gillet1, Jorge S Valadas1

  • 1Neuroinflammation Focus Area, Neuroscience Research, UCB Biopharma SRL, Braine-l'Alleud, Belgium.

Frontiers in Molecular Neuroscience
|July 7, 2023
PubMed
Summary

Huntington's disease (HD) involves early immune cell activation and microglial changes impacting synapses. Targeting these microglial functions may offer new therapeutic strategies for HD cognitive and psychiatric symptoms.

Keywords:
Huntington’s diseaseR6/2 modelcyTOFdendritic spinesmicrogliamigrationneuroinflammationphagocytosis

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Area of Science:

  • Neuroscience
  • Immunology
  • Genetics

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by CAG repeat expansion in the HTT gene.
  • Alterations in neuronal circuitry and synaptic loss are hallmarks of HD, alongside immune system involvement.
  • The precise role of microglial and peripheral immune activation in HD pathogenesis and synaptic health remains unclear.

Purpose of the Study:

  • To investigate immune phenotypes and functional activation states of microglia and peripheral immunity in a mouse model of HD across disease stages.
  • To characterize microglial morphology, surveillance, and phagocytosis, and their impact on synaptic loss.
  • To assess the relevance of observed microglial behaviors to human HD through transcriptomic and iPSC-derived cell analyses.

Main Methods:

  • Utilized the R6/2 mouse model of HD for pre-symptomatic, symptomatic, and end-stage analyses.
  • Performed single-cell resolution characterization of microglial phenotypes, morphology, and functions (surveillance, phagocytosis).
  • Conducted transcriptomic analysis on HD patient data and functional assessments using induced pluripotent stem cell (iPSC)-derived microglia.

Main Results:

  • Observed temporal changes including peripheral immune cell infiltration and increased microglial activation and phagocytosis in pre-symptomatic HD.
  • Found increased microglial surveillance and synaptic uptake correlating with reduced spine density in R6/2 mice.
  • Identified upregulated endocytic and migratory gene pathways in human HD microglia and enhanced phagocytic/migratory functions in iPSC-derived HD microglia.

Conclusions:

  • Aberrant microglial functions, including synaptic surveillance and phagocytosis, are evident early in HD pathogenesis.
  • These microglial changes are conserved in human HD and correlate with synaptic loss.
  • Targeting specific microglial functions may represent a promising therapeutic avenue for mitigating HD-related cognitive and psychiatric symptoms.