GFAP Mutations in Astrocytes Impair Oligodendrocyte Progenitor Proliferation and Myelination in an hiPSC Model of Alexander Disease

Shi and colleagues used Alexander disease (AxD) patient iPSC-derived astrocytes to recapitulate AxD patient phenotypes that could not be achieved in animal models and uncover molecular mechanisms underlying myelination defect in the disease. They found that disease astrocytes secret molecules to inhibit oligodendrocyte progenitor cell function and impair myelination.

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