Gal3 suppression delays the motor coordination loss in the ataxic tambaleante mouse model
Rocío Ruiz, Francisco Hernández-Rasco, Alberto Rivera-Ramos, Isabel M. Alonso-Bellido, Maria A. Roca-Ceballos, Irene García-Domínguez, María S. Letrán-Sánchez, Jesús Soldán-Hidalgo, Sara Bachiller, Ana M. Espinosa-Oliva, Rocío M. de Pablos, Eva M. Pérez-Villegas, Antonio J. Herrera, José A. Armengol, José L. Venero · FENS Forum 2024
The autosomal recessive mutation Gly483Glu, occurring spontaneously in the murine protein Herc1, leads to its overexpression in the cerebellum of the tambaleante mouse (tbl). This causes dysregulation of autophagy-mediated death of Purkinje cells (PC) from two months of age, along with dysregulation of the PI3K/AKT/mTOR signalling pathway responsible for proper axonal myelination. Both situations trigger the onset of an ataxic neuromuscular syndrome characterized by motor control impairments. In response to this disturbance, even before PC death occurs, microglia present in the cerebellum can
ePoster · Neuroscience