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Publication : Retinal Tissue Shows Glial Changes in a Dravet Syndrome Knock-in Mouse Model.

First Author  Salazar JJ Year  2023
Journal  Int J Mol Sci Volume  24
Issue  3 PubMed ID  36769051
Mgi Jnum  J:333507 Mgi Id  MGI:7436420
Doi  10.3390/ijms24032727 Citation  Salazar JJ, et al. (2023) Retinal Tissue Shows Glial Changes in a Dravet Syndrome Knock-in Mouse Model. Int J Mol Sci 24(3)
abstractText  Dravet syndrome (DS) is an epileptic encephalopathy caused by mutations in the Scn1a gene encoding the alpha1 subunit of the Nav1.1 sodium channel, which is associated with recurrent and generalized seizures, even leading to death. In experimental models of DS, histological alterations have been found in the brain; however, the retina is a projection of the brain and there are no studies that analyze the possible histological changes that may occur in the disease. This study analyzes the retinal histological changes in glial cells (microglia and astrocytes), retinal ganglion cells (RGCs) and GABAergic amacrine cells in an experimental model of DS (Syn-Cre/Scn1a(WT/A1783V)) compared to a control group at postnatal day (PND) 25. Retinal whole-mounts were labeled with anti-GFAP, anti-Iba-1, anti-Brn3a and anti-GAD65/67. Signs of microglial and astroglial activation, and the number of Brn3a+ and GAD65+67+ cells were quantified. We found retinal activation of astroglial and microglial cells but not death of RGCs and GABAergic amacrine cells. These changes are similar to those found at the level of the hippocampus in the same experimental model in PND25, indicating a relationship between brain and retinal changes in DS. This suggests that the retina could serve as a possible biomarker in DS.
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