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First published online August 17, 2004
doi: 10.1242/10.1242/jcs.01301

Journal of Cell Science 117, 4169-4177 (2004)
Published by The Company of Biologists 2004
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Research Article

Crumbs homologue 1 is required for maintenance of photoreceptor cell polarization and adhesion during light exposure

Serge A. van de Pavert1,*, Albena Kantardzhieva1,*, Anna Malysheva1, Jan Meuleman1, Inge Versteeg1, Christiaan Levelt1, Jan Klooster1, Sylvia Geiger2, Mathias W. Seeliger2, Penny Rashbass3, Andre Le Bivic4 and Jan Wijnholds1,{ddagger}

1 The Netherlands Ophthalmic Research Institute, Royal Netherlands Academy of Arts and Sciences, Meibergdreef 47, 1105 BA Amsterdam, The Netherlands
2 Retinal Electrodiagnostics Research Group, Department of Ophthalmology, University of Tübingen, Schleichstr. 12-16, 72076 Tübingen, Germany
3 Centre for Developmental Genetics, Department of Biomedical Science, University of Sheffield, Western Bank, Sheffield, S10 2TN, UK
4 Laboratoire de Neurogenèse et Morphogenèse au cours du Développement et chez l'Adulte (NMDA), UMR 6156 CNRS, IBDM, Université de la Méditerranée, Campus de Luminy, case 907, 13288 Marseille CEDEX 09, France

{ddagger} Author for correspondence (e-mail: j.wijnholds{at}ioi.knaw.nl)

Accepted 4 May 2004

Loss of Crumbs homologue 1 (CRB1) function causes either the eye disease Leber congenital amaurosis or progressive retinitis pigmentosa, depending on the amount of residual CRB1 activity and the genetic background. Crb1 localizes specifically to the sub-apical region adjacent to the adherens junction complex at the outer limiting membrane in the retina. We show that it is associated here with multiple PDZ protein 1 (Mupp1), protein associated with Lin-7 (Pals1 or Mpp5) and Mpp4. We have produced Crb1-/- mice completely lacking any functional Crb1. Although the retinas are initially normal, by 3-9 months the Crb1-/- retinas develop localized lesions where the integrity of the outer limiting membrane is lost and giant half rosettes are formed. After delamination of the photoreceptor layer, neuronal cell death occurs in the inner and outer nuclear layers of the retina. On moderate exposure to light for 3 days at 3 months of age, the number of severe focal retinal lesions significantly increases in the Crb1-/- retina. Crb2, Crb3 and Crb1 interacting proteins remain localized to the sub-apical region and therefore are not sufficient to maintain cell adhesion during light exposure in Crb1-/- retinas. Thus we propose that during light exposure Crb1 is essential to maintain, but not assemble, adherens junctions between photoreceptors and Müller glia cells and prevents retinal disorganization and dystrophy. Hence, light may be an influential factor in the development of the corresponding human diseases.

Key words: Leber congenital amaurosis, Light induced degeneration, Cell polarity, Cell adhesion, Subapical region, Adherens junction


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