Phenotypical changes of satellite glial cells in a murine model of G(M1)‐gangliosidosis

Satellite glial cells (SGCs) of dorsal root ganglia (DRG) react in response to various injuries in the nervous system. This study investigates reactive changes within SGCs in a murine model for G(M1)‐gangliosidosis (G(M1)). DRG of homozygous β‐galactosidase‐knockout mice and homozygous C57BL/6 wild‐type mice were investigated performing immunostaining on formalin‐fixed, paraffin‐embedded tissue. A marked upregulation of glial fibrillary acidic protein (GFAP), the progenitor marker nestin and Ki67 within SGCs of diseased mice, starting after 4 months at the earliest GFAP, along with intracytoplasmic accumulation of ganglioside within neurons and deterioration of clinical signs was identified. Interestingly, nestin‐positive SGCs were detected after 8 months only. No changes regarding inwardly rectifying potassium channel 4.1, 2, 3‐cyclic nucleotide 3‐phosphodiesterase, Sox2, doublecortin, periaxin and caspase3 were observed in SGCs. Iba1 was only detected in close vicinity of SGCs indicating infiltrating or tissue‐resident macrophages. These results indicate that SGCs of DRG show phenotypical changes during the course of G(M1), characterized by GFAP upregulation, proliferation and expression of a neural progenitor marker at a late time point. This points towards an important role of SGCs during neurodegenerative disorders and supports that SGCs represent a multipotent glial precursor cell line with high plasticity and functionality.