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Prog Retin Eye Res. 2014 May;40:35-52. doi: 10.1016/j.preteyeres.2014.01.001. Epub 2014 Feb 6.

The CRB1 and adherens junction complex proteins in retinal development and maintenance.

Author information

1
Department of Neuromedical Genetics, The Netherlands Institute for Neuroscience, Royal Netherlands Academy of Arts and Sciences (KNAW), Meibergdreef 47, 1105 BA Amsterdam, The Netherlands.
2
Department of Neuromedical Genetics, The Netherlands Institute for Neuroscience, Royal Netherlands Academy of Arts and Sciences (KNAW), Meibergdreef 47, 1105 BA Amsterdam, The Netherlands. Electronic address: j.wijnholds@nin.knaw.nl.

Abstract

The early developing retinal neuroepithelium is composed of multipotent retinal progenitor cells that differentiate in a time specific manner, giving rise to six major types of neuronal and one type of glial cells. These cells migrate and organize in three distinct nuclear layers divided by two plexiform layers. Apical and adherens junction complexes have a crucial role in this process by the establishment of polarity and adhesion. Changes in these complexes disturb the spatiotemporal aspects of retinogenesis, leading to retinal degeneration resulting in mild or severe impairment of retinal function and vision. In this review, we summarize the mouse models for the different members of the apical and adherens junction protein complexes and describe the main features of their retinal phenotypes. The knowledge acquired from the different mutant animals for these proteins corroborate their importance in retina development and maintenance of normal retinal structure and function. More recently, several studies have tried to unravel the connection between the apical proteins, important cellular signaling pathways and their relation in retina development. Still, the mechanisms by which these proteins function remain largely unknown. Here, we hypothesize how the mammalian apical CRB1 complex might control retinogenesis and prevents onset of Leber congenital amaurosis or retinitis pigmentosa.

KEYWORDS:

Apical protein complexes; CRB1 complex; Leber congenital amaurosis; Neuroepithelium; Retina; Retinitis pigmentosa

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