Functional Abnormalities of Cerebellum and Motor Cortex in Spinal Muscular Atrophy Mice.

ABSTRACT: Spinal muscular atrophy (SMA) is a devastating genetic neuromuscular disease. Diffuse neuropathology has been reported in SMA patients and mouse models, however, functional changes in brain regions have not been studied. In the SMNΔ7 mouse model, we identified three types of differences in neuronal function in the cerebellum and motor cortex from two age groups: P7-9 (P7) and P11-14 (P11). Microelectrode array studies revealed significantly lower spontaneous firing and network activity in the cerebellum of SMA mice in both age groups, but it was more profound in the P11 group. In the motor cortex, however, neural activity was not different in either age group. Whole-cell patch-clamp was used to study the function of output neurons in both brain regions. In cerebellar Purkinje cells (PCs) o

SUBMITTER: Tharaneetharan A 

PROVIDER: S-EPMC7771387 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature