Purkinje Cell-specific Deficiency in SEL1L-HRD1 Endoplasmic Reticulum-Associated Degradation Causes Progressive Cerebellar Ataxia in Mice
Mauricio Torres, Hui Wang, Brent Pederson, Liangguang Leo Lin, Huilun Helen Wang, Amara Bugarin-Lapuz, Zhen Zhao, Ling Qi
This study shows that SEL1L-HRD1 ERAD is essential for Purkinje cell function and causes progressive cerebellar ataxia in mice.
JCI Insight · Oct 1, 2024Peer-reviewedNeuroscience