Specific ZNF274 binding interference at SNORD116 activates the maternal transcripts in Prader-Willi syndrome neurons
Maéva Langouët, Dea Gorka, Clarisse Orniacki, Clémence M Dupont-Thibert, Michael S Chung, Heather R. Glatt-Deeley, Noélle D. Germain, Leann J Crandall, Justin L. Cotney, Christopher E. Stoddard, Marc Lalande, Stormy J. Chamberlain
The paper shows that disrupting ZNF274 binding at SNORD116 activates its maternal allele in PWS neurons without altering ZNF274 levels, suggesting a therapeutic strategy for Prader-Willi syndrome.
Human Molecular Genetics · Sep 18, 2020Peer-reviewedGenetics