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More importantly, using human embryonic stem cell (hESC) culture models, researchers showed that these mitochondrial dysfunction signatures occur in HD-affected hESCs but not HD-affected hESCs-derived neural lineage cells, while a shift to transcriptional dysregulation and cytoskeletal abnormalities, the primary HD pathologies, appears in HD-affected differentiating hESCs along neural lineages in vitro (Monk and Connor, 2021)

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