Age-related Huntington’s disease progression modeled in directly reprogrammed patient-derived striatal neurons highlights impaired autophagy

• Published in: Nature neuroscience Vol. 25; no. 11; pp. 1420 - 1433
• Main Authors: Oh, Young Mi, Lee, Seong Won, Kim, Woo Kyung, Chen, Shawei, Church, Victoria A., Cates, Kitra, Li, Tiandao, Zhang, Bo, Dolle, Roland E., Dahiya, Sonika, Pak, Stephen C., Silverman, Gary A., Perlmutter, David H., Yoo, Andrew S.
• Format: Journal Article
• Language: English
• Published: New York Nature Publishing Group US 01.11.2022; Nature Publishing Group
• Subjects: 3' Untranslated regions; 631/378/1689/1558; 631/378/1689/364; 631/378/2611; 631/80/39; Age; Aging; Animal Genetics and Genomics; Animals; Autophagy; Behavioral Sciences; Biological Techniques; Biomedical and Life Sciences; Biomedicine; Chromatin; Corpus Striatum - physiology; Degeneration; Disease Models, Animal; Disease Progression; Fibroblasts; Humans; Huntington Disease - pathology; Huntington's disease; Huntingtons disease; MicroRNAs - genetics; miRNA; Neostriatum; Neurobiology; Neurodegeneration; Neurodegenerative diseases; Neurons; Neurons - physiology; Neurosciences; Patients; Resilience; Signs and symptoms; Spiny neurons; Stat3 protein

Huntington’s disease (HD) is an inherited neurodegenerative disorder with adult-onset clinical symptoms, but the mechanism by which aging drives the onset of neurodegeneration in patients with HD remains unclear. In this study we examined striatal medium spiny neurons (MSNs) directly reprogrammed fr...