Activation of XBP1s attenuates disease severity in models of proteotoxic Charcot-Marie-Tooth type 1B
Thierry Touvier, Francesca A Veneri, Anke Claessens, Cinzia Ferri, R Mastrangelo, Noémie Sorgiati, Francesca Bianchi, Serena Valenzano, Ubaldo Del Carro, Cristina Rivellini, Phu Duong, Michael E. Shy, Jeffery W. Kelly, John P. Svaren, R. Luke Wiseman, Maurizio D’Antonio
This study shows that activating XBP1s reduces disease severity in CMT1B models by mitigating ER stress and mutant protein toxicity in Schwann cells.
Brain · Dec 20, 2024Peer-reviewedNeuroscience