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

Dravet syndrome (DS) is an epileptic encephalopathy caused by mutations in the Scn1a gene encoding the α1 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 i...

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Detalles Bibliográficos
Autores: Salazar Corral, Juan José, Satriano, Andrea, Matamoros, José A., Fernández Albarral, José, García Martín, Elena Salobrar, López Cuenca, Inés, Hoz Montañana, María Rosa De, Sánchez-Puebla Fernández, Lidia, Ramírez Sebastián, José Manuel, Alonso, Cristina, Satta, Valentina, Hernández Fisac, Inés, Sagredo Ezquioga, Onintza, Ramírez Sebastián, Ana Isabel
Tipo de recurso: artículo
Fecha de publicación:2023
País:España
Institución:Universidad Complutense de Madrid (UCM)
Repositorio:Docta Complutense
Idioma:inglés
OAI Identifier:oai:docta.ucm.es:20.500.14352/72989
Acceso en línea:https://hdl.handle.net/20.500.14352/72989
Access Level:acceso abierto
Palabra clave:616-056.7
611.8.018.24
617.735
Dravet syndrome
Retina
Astrocytes
Microglia
Retinal ganglion cells
GABAergic amacrine cells
Syn-Cre/Scn1aWT/A1783V mice
Neurociencias (Medicina)
Oftalmología
Anatomía ocular
2490 Neurociencias
3201.09 Oftalmología
Descripción
Sumario:Dravet syndrome (DS) is an epileptic encephalopathy caused by mutations in the Scn1a gene encoding the α1 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/Scn1aWT/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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