Activation of XBP1s attenuates disease severity in models of proteotoxic Charcot–Marie–Tooth type 1B - Touvier, Thierry; Veneri, Francesca A; Claessens, Anke; Ferri, Cinzia; Mastrangelo, Rosa; Sorgiati, Noémie; Bianchi, Francesca; Valenzano, Serena; Del Carro, Ubaldo; Rivellini, Cristina; Duong, Phu; Shy, Michael E; et al. (Brain (London, England : 1878), 2025)
Keywords: Animals; Charcot-Marie-Tooth Disease - genetics; Charcot-Marie-Tooth Disease - metabolism; Charcot-Marie-Tooth Disease - pathology; Disease Models, Animal; Endoplasmic Reticulum Stress - physiology; Endoribonucleases - metabolism; Mice; Mice, Inbred C57BL; Mice, Transgenic; Myelin P0 Protein - genetics; Myelin P0 Protein - metabolism; Myelin Sheath - metabolism; Original; Protein Serine-Threonine Kinases - metabolism; Schwann Cells - metabolism; Unfolded Protein Response; X-Box Binding Protein 1 - genetics; X-Box Binding Protein 1 - metabolism; unfolded protein response; Charcot–Marie–Tooth; XBP1; proteostasis; Schwann cell; demyelinating neuropathy