RT Journal Article
T1 Retinal Tissue Shows Glial Changes in a Dravet Syndrome Knock-in Mouse Model
A1 Salazar Corral, Juan José
A1 Satriano, Andrea
A1 Matamoros, José A.
A1 Fernández Albarral, José
A1 García Martín, Elena Salobrar
A1 López Cuenca, Inés
A1 Hoz Montañana, María Rosa De
A1 Sánchez-Puebla Fernández, Lidia
A1 Ramírez Sebastián, José Manuel
A1 Alonso, Cristina
A1 Satta, Valentina
A1 Hernández Fisac, Inés
A1 Sagredo Ezquioga, Onintza
A1 Ramírez Sebastián, Ana Isabel
AB 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.
PB MDPI
SN 1422-0067
YR 2023
FD 2023-02-01
LK https://hdl.handle.net/20.500.14352/72989
UL https://hdl.handle.net/20.500.14352/72989
LA eng
NO Salazar, Juan J., Satriano, A., Matamoros, J. A. et al. «Retinal Tissue Shows Glial Changes in a Dravet Syndrome Knock-in Mouse Model». International Journal of Molecular Sciences, vol. 24, n.o 3, febrero de 2023, p. 2727. DOI.org (Crossref), https://doi.org/10.3390/ijms24032727.
NO Ministerio de Ciencia, Innovación y Universidades (España)
NO Instituto de Salud Carlos III
NO Universidad Complutense de Madrid
DS Docta Complutense
RD 6 abr 2025