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Phenotypical changes of satellite glial cells in a murine model of GM1 -gangliosidosis.


ABSTRACT: 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 GM1 -gangliosidosis (GM1 ). 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 GM1 , 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.

SUBMITTER: Huang B 

PROVIDER: S-EPMC8743646 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature

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Phenotypical changes of satellite glial cells in a murine model of G<sub>M1</sub> -gangliosidosis.

Huang Bei B   Zdora Isabel I   de Buhr Nicole N   Eikelberg Deborah D   Baumgärtner Wolfgang W   Leitzen Eva E  

Journal of cellular and molecular medicine 20211207 2


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<sub>M1</sub> -gangliosidosis (G<sub>M1</sub> ). 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 m  ...[more]

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