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Brain immune cells at the centre of white matter disease pathology

The Hamilton’s NeuroImmunology in Action Lab uses the zebrafish as a model to study the intricate and dynamic behaviour of the brain’s immune cells, the microglia. Dr. Noémie Hamilton's research investigates the mechanistic roles of microglial dysfunction in genetic white matter disorders and explores targeted cellular therapies to restore Central Nervous System (CNS) homeostasis.

  • Microglial Phagocytic Failure in RNASET2 Deficiency: Early work identified that RNASET2 deficiency impairs the capacity of microglia to clear developmental apoptotic cells, triggering neuroinflammation and driving the progression of RNASET2-deficient leukoencephalopathy. (Hamilton et al, Glia 2020)
  • Therapeutic Rescue via Macrophage Transplantation: Building on these disease mechanisms, subsequent studies demonstrated that transplanting functional macrophages into microglia-depleted host models successfully populates the microglial niche, restores apoptotic cell clearance, and rescues the leukodystrophy phenotype. (Rutherford et al, PNAS 2024)
  • Genetic Spectrum & Neuroinflammation in SUPV3L1 Mutations: Expanding into novel genetic causes, recent findings define SUPV3L1 biallelic mutations as a source of variable leukodystrophy, linking impaired mitochondrial degradosome function and dysplastic microglia to the activation of the type-1 interferon innate immune pathway. (Green and Hamilton, et al ResearchSquare 2026)

 Lead researcher