Human ESC-Derived Chimeric Mouse Models of Huntington’s Disease Reveal Cell-Intrinsic Defects in Glial Progenitor Cell Differentiation

The authors show that glial progenitor cells derived from human ESCs expressing mutant Huntingtin (mHTT) are delayed and defective in their maturation; their suppressed transcription of differentiation-associated genes leads to both astrocytic dysfunction and myelin deficiency in vivo. Glial pathology may thus contribute to disease phenotype in Huntington's disease.

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