RT Journal Article
T1 Tlr2 Gene Deletion Delays Retinal Degeneration in Two Genetically Distinct Mouse Models of Retinitis Pigmentosa
A1 Sánchez Cruz, Alonso
A1 Canto Méndez, Andrea
A1 Lizasoaín Hernández, Ignacio
A1 de la Villa, Pedro
A1 Rosa, Enrique J. De La
A1 Hernández Sánchez, Catalina
AB Although considered a rare retinal dystrophy, retinitis pigmentosa (RP) is the primary cause of hereditary blindness. Given its diverse genetic etiology (>3000 mutations in >60 genes), there is an urgent need for novel treatments that target common features of the disease. TLR2 is a key activator of innate immune response. To examine its role in RP progression we characterized the expression profile of Tlr2 and its adaptor molecules and the consequences of Tlr2 deletion in two genetically distinct models of RP: Pde6brd10/rd10 (rd10) and RhoP23H/+ (P23H/+) mice. In both models, expression levels of Tlr2 and its adaptor molecules increased in parallel with those of the proinflammatory cytokine Il1b. In rd10 mice, deletion of a single Tlr2 allele had no effect on visual function, as evaluated by electroretinography. However, in both RP models, complete elimination of Tlr2 attenuated the loss of visual function and mitigated the loss of photoreceptor cell numbers. In Tlr2 null rd10 mice, we observed decreases in the total number of microglial cells, assessed by flow cytometry, and in the number of microglia infiltrating the photoreceptor layers. Together, these results point to TLR2 as a mutation-independent therapeutic target for RP.
PB MPDI
SN 1422-0067
YR 2021
FD 2021-06-22
LK https://hdl.handle.net/20.500.14352/7056
UL https://hdl.handle.net/20.500.14352/7056
LA eng
NO Sánchez Cruz, A., Canto Méndez, A., Lizasoaín Hernández, I. et al. «Tlr2 Gene Deletion Delays Retinal Degeneration in Two Genetically Distinct Mouse Models of Retinitis Pigmentosa». International Journal of Molecular Sciences, vol. 22, n.o 15, julio de 2021, p. 7815. DOI.org (Crossref), https://doi.org/10.3390/ijms22157815.
NO Ministerio de Economía, Comercio y Empresa (España)
NO Instituto de Salud Carlos III/Fondo Europeo de Desarrollo Regional
NO Comunidad de Madrid
DS Docta Complutense
RD 10 may 2025