Underdeveloped RPE Apical Domain Underlies Lesion Formation in Canine Bestrophinopathies.

Autor:	Guziewicz KE; Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA. karinag@vet.upenn.edu.; University of Pennsylvania, Ryan Veterinary Hospital, Philadelphia, PA, USA. karinag@vet.upenn.edu., McTish E; Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA., Dufour VL; Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA., Zorych K; Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA., Dhingra A; University of Pennsylvania, Ryan Veterinary Hospital, Philadelphia, PA, USA., Boesze-Battaglia K; Department of Biochemistry, School of Dental Medicine, University of Pennsylvania, Philadelphia, PA, USA., Aguirre GD; Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Jazyk:	angličtina
Zdroj:	Advances in experimental medicine and biology [Adv Exp Med Biol] 2018; Vol. 1074, pp. 309-315.
DOI:	10.1007/978-3-319-75402-4_38
Abstrakt:	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.
Databáze:	MEDLINE
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