Tlr2 Gene Deletion Delays Retinal Degeneration in Two Genetically Distinct Mouse Models of Retinitis Pigmentosa
نویسندگان
چکیده
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 an urgent need for novel treatments that target common features disease. TLR2 key activator innate immune response. To examine role RP progression we characterized expression profile Tlr2 and adaptor molecules consequences deletion two genetically distinct models RP: Pde6brd10/rd10 (rd10) RhoP23H/+ (P23H/+) mice. In both models, levels increased parallel with those proinflammatory cytokine Il1b. rd10 mice, single allele had no effect on visual function, as evaluated by electroretinography. However, complete elimination attenuated loss function mitigated photoreceptor cell numbers. null observed decreases total number microglial cells, assessed flow cytometry, microglia infiltrating layers. Together, these results point to mutation-independent therapeutic RP.
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ژورنال
عنوان ژورنال: International Journal of Molecular Sciences
سال: 2021
ISSN: ['1661-6596', '1422-0067']
DOI: https://doi.org/10.3390/ijms22157815