Guziewicz, Karina E.McTish, EmilyDufour, Valerie L.Zorych, KathrynDhingra, AnuradhaBoesze-Battaglia, KathleenAguirre, Gustavo D.2023-05-222023-05-222018-01-012020-12-07https://repository.upenn.edu/handle/20.500.14332/8919Canine bestrophinopathy (cBest) is an important translational model for BEST1-associated maculopathies in man that recapitulates the broad spectrum of clinical and molecular disease aspects observed in patients. Both human and canine bestrophinopathies are characterized by focal to multifocal separations of the retina from the RPE. The lesions can be macular or extramacular, and the specific pathomechanism leading to formation of these lesions remains unclear. We used the naturally occurring canine BEST1 model to examine factors that underlie formation of vitelliform lesions and addressed the susceptibility of the macula to its primary detachment in BEST1-linked maculopathies.This is a post-peer-review, pre-copyedit version of an article published in Advances in Experimental Medicine and Biology. The final authenticated version is available online at: http://dx.doi.org/10.1007/978-3-319-75402-4_38BEST1BestrophinopathiesCanine modelMaculaVitelliform lesion formationRPE-photoreceptor interfaceRPE microvilliCone photoreceptorsRPE apical domainInsoluble interphotoreceptor matrixDentistryArticle