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DRG of homozygous \u03b2\u2010galactosidase\u2010knockout mice and homozygous C57BL\/6 wild\u2010type mice were investigated performing immunostaining on formalin\u2010fixed, paraffin\u2010embedded tissue. A marked upregulation of glial fibrillary acidic protein (GFAP), the progenitor marker nestin and Ki67 within SGCs of diseased mice, starting after 4\u00a0months at the earliest GFAP, along with intracytoplasmic accumulation of ganglioside within neurons and deterioration of clinical signs was identified. Interestingly, nestin\u2010positive SGCs were detected after 8\u00a0months only. No changes regarding inwardly rectifying potassium channel 4.1, 2, 3\u2010cyclic nucleotide 3\u2010phosphodiesterase, Sox2, doublecortin, periaxin and caspase3 were observed in SGCs. Iba1 was only detected in close vicinity of SGCs indicating infiltrating or tissue\u2010resident macrophages. These results indicate that SGCs of DRG show phenotypical changes during the course of G<jats:sub>M1<\/jats:sub>, 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.<\/jats:p>","DOI":"10.1111\/jcmm.17113","type":"journal-article","created":{"date-parts":[[2021,12,8]],"date-time":"2021-12-08T07:30:41Z","timestamp":1638948641000},"page":"527-539","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Phenotypical changes of satellite glial cells in a murine model of G<sub>M1<\/sub>\u2010gangliosidosis"],"prefix":"10.1111","volume":"26","author":[{"given":"Bei","family":"Huang","sequence":"first","affiliation":[{"name":"Department of Pathology University of Veterinary Medicine Hannover Hannover Germany"},{"name":"Center of Systems Neuroscience Hannover 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