Underdeveloped RPE Apical Domain Underlies Lesion Formation in Canine Bestrophinopathies

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Departmental Papers (Dental)
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BEST1
Bestrophinopathies
Canine model
Macula
Vitelliform lesion formation
RPE-photoreceptor interface
RPE microvilli
Cone photoreceptors
RPE apical domain
Insoluble interphotoreceptor matrix
Dentistry
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Guziewicz, Karina E.
McTish, Emily
Dufour, Valerie L.
Zorych, Kathryn
Dhingra, Anuradha
Boesze-Battaglia, Kathleen
Aguirre, Gustavo D.
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Abstract

Canine 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.

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2018-01-01
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Advances in Experimental Medicine and Biology
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