Structure-function dissection of huntingtin exon 1 identifies a PRD-driven modifier of neuronal toxicity in Huntington's disease
Raffaele Iennaco, Camilla Maffezzini, Simone Maestri, Andrea Scolz, Angela Cattaneo, Alessio Murgia, Camilla Trovesi, Eugenia Cammarota, Elena Vezzoli, Andrea Falqui, Dan P. Felsenfeld, Thomas Vogt, Angela Bachi, Chiara Zuccato, Elena F. Cattaneo
The paper identifies the proline-rich domain (PRD) of HTT exon 1 as a modifier of neuronal toxicity in Huntington's disease, showing that the human PRD exacerbates while the mouse PRD mitigates toxicity.
bioRxiv · Jul 27, 2026 · PreprintNeuroscience