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Generation and Characterization of Human Induced Pluripotent Stem Cell-derived Astrocytes Lacking Fragile X Messenger Ribonucleoprotein
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Stem-Cell-Derived Astrocytes Divulge Secrets of Mutant GFAP
1Department of Neurobiology, University of California Los Angeles, Los Angeles, CA, USA.
Abstract:
Gain-of-function mutations in the canonical astrocyte protein, GFAP, cause a fatal neurodevelopmental disorder with myelination abnormalities, seizures, and psychomotor disturbances. Recently, Li et al. (2018) (in Cell Stem Cell) and Jones et al. (2018) (in Cell Reports) have shown that patient iPSC-derived astrocytes with mutant GFAP have disrupted astrocyte functions, revealing disease mechanisms and potential roles of GFAP.
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