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Biol Open. 2016 Nov 15;5(11):1662-1673. doi: 10.1242/bio.021584.

Eyes shut homolog is required for maintaining the ciliary pocket and survival of photoreceptors in zebrafish.

Author information

1
Center for Vision Research, Departments of Ophthalmology and Neuroscience and Physiology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
2
Department of Public Health and Preventive Medicine, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
3
Department of Cell and Developmental Biology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
4
Center for Vision Research, Departments of Ophthalmology and Neuroscience and Physiology, SUNY Upstate Medical University, Syracuse, NY 13210, USA huh@upstate.edu.

Abstract

Mutations in the extracellular matrix protein eyes shut homolog (EYS) cause photoreceptor degeneration in patients with retinitis pigmentosa 25 (RP25). Functions of EYS remain poorly understood, due in part to the lack of an EYS gene in mouse. We investigated the localization of vertebrate EYS proteins and engineered loss-of-function alleles in zebrafish. Immunostaining indicated that EYS localized near the connecting cilium/transition zone in photoreceptors. EYS also strongly localized to the cone outer segments and weakly to the rod outer segments and cone terminals in primate retinas. Analysis of mutant EYS zebrafish revealed disruption of the ciliary pocket in cone photoreceptors, indicating that EYS is required for maintaining the integrity of the ciliary pocket lumen. Mutant zebrafish exhibited progressive loss of cone and rod photoreceptors. Our results indicate that EYS protein localization is species-dependent and that EYS is required for maintaining ciliary pocket morphology and survival of photoreceptors in zebrafish.

KEYWORDS:

Connecting cilia; Degeneration; Extracellular matrix; Photoreceptors; Retinal degeneration; Retinitis pigmentosa

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