Recent studies have uncovered a role for astrocytes in the pathophysiology of Rett syndrome, most cases of which are linked to mutations in methyl-CpG-binding protein 2 (MECP2). A new study shows that microglial dysfunction may also have an important role in this disorder. Transplantation of wild-type murine bone marrow into male Mecp2−/− mice that had undergone postnatal irradiation led to engraftment of MECP2-expressing microglia-like cells in the brain parenchyma and a cessation of disease progression. Moreover, mice in which MECP2 expression was restricted to myeloid cells, including microglia, showed a similar phenotype. Finally, pharmacological blockade of microglial phagocytic activity in these animals attenuated disease improvement.
ORIGINAL RESEARCH PAPER
Derecki, N. C. et al. Wild-type microglia arrest pathology in a mouse model of Rett syndrome. Nature 18 Mar 2012 (doi:10.1038/nature10907)
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Yates, D. Microglia — major players in Rett syndrome?. Nat Rev Neurosci 13, 291 (2012). https://doi.org/10.1038/nrn3237
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DOI: https://doi.org/10.1038/nrn3237