Aberrant Development Corrected in Adult-Onset Huntington's Disease iPSC-Derived Neuronal Cultures via WNT Signaling Modulation
Author(s)
Smith-Geater, Charlene; Hernandez, Sarah J; Lim, Ryan G; Adam, Miriam; Wu, Jie; Stocksdale, Jennifer T; Wassie, Brook T; Gold, Maxwell Philip; Wang, Keona Q; Miramontes, Ricardo; Kopan, Lexi; Orellana, Iliana; Joy, Shona; Kemp, Paul J; Allen, Nicholas D; Fraenkel, Ernest; Thompson, Leslie M; ... Show more Show less
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© 2020 The Authors Thompson et al. describe a cyclin D1+ neural stem cell population unique to adult-onset Huntington's disease iPSC-derived neuronal cultures, differentiated using a protocol that increases the derivation of medium spiny neurons. These findings highlight a selective CAG-length-dependent deficit in neurodevelopment. This aberrant mitotic population is ameliorated with WNT inhibition, perhaps identifying a mechanism that can be exploited for therapeutic compensation.
Date issued
2020Department
Massachusetts Institute of Technology. Department of Biological EngineeringJournal
Stem Cell Reports
Publisher
Elsevier BV