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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Acknowledgments
This study was supported by FFB-TRAP and FFB-Facility grants, MVRF, NEI/NIH EY06855, EY10420, and Hope for Vision.
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Guziewicz, K.E. et al. (2018). Underdeveloped RPE Apical Domain Underlies Lesion Formation in Canine Bestrophinopathies. In: Ash, J., Anderson, R., LaVail, M., Bowes Rickman, C., Hollyfield, J., Grimm, C. (eds) Retinal Degenerative Diseases. Advances in Experimental Medicine and Biology, vol 1074. Springer, Cham. https://doi.org/10.1007/978-3-319-75402-4_38
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DOI: https://doi.org/10.1007/978-3-319-75402-4_38
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