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A model of astrocyte pathology in TDP-43 proteinopathies. Astrocytes derived from human iPSCs are used to examine the cell-autonomous effects of TDP-43 mislocalization. Astrocytes that are generated from patients harboring ALS-causing mutations in the TARDBP gene display cytoplasmic TDP-43 inclusions, mimicking the common neuronal and glial histopathologic findings. Mutant TDP-43 patient-derived astrocytes display significantly impaired survival after 10 d.
