Guanabenz treatment improves Oculopharyngeal muscular dystrophy phenotype
A. Malerba, F. Roth, P. Harish, J. Dhiab, N. Lu-Nguyen, O. Cappellari, S. Jarmin, A. Mahoudeau, V. Ythier, J. Laine, E. Negroni, E. Abgueguen, M. Simonelig, P. Guedat, V. Mouly, , C. Trollet
This study shows that guanabenz acetate reduces PABPN1 aggregates and improves muscle function in an OPMD mouse model by modulating the UPR.
bioRxiv · Jul 24, 2018 · PreprintPathology