Ontology highlight
ABSTRACT:
SUBMITTER: Bowie LE
PROVIDER: S-EPMC6064984 | biostudies-literature | 2018 Jul
REPOSITORIES: biostudies-literature
Bowie Laura E LE Maiuri Tamara T Alpaugh Melanie M Gabriel Michelle M Arbez Nicolas N Galleguillos Danny D Hung Claudia L K CLK Patel Shreya S Xia Jianrun J Hertz Nicholas T NT Ross Christopher A CA Litchfield David W DW Sipione Simonetta S Truant Ray R
Proceedings of the National Academy of Sciences of the United States of America 20180709 30
The huntingtin N17 domain is a modulator of mutant huntingtin toxicity and is hypophosphorylated in Huntington's disease (HD). We conducted high-content analysis to find compounds that could restore N17 phosphorylation. One lead compound from this screen was N6-furfuryladenine (N6FFA). N6FFA was protective in HD model neurons, and N6FFA treatment of an HD mouse model corrects HD phenotypes and eliminates cortical mutant huntingtin inclusions. We show that N6FFA restores N17 phosphorylation level ...[more]