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Human Molecular Genetics Advance Access originally published online on August 2, 2006
Human Molecular Genetics 2006 15(18):2659-2672; doi:10.1093/hmg/ddl194
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© The Author 2006. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Pals1/Mpp5 is required for correct localization of Crb1 at the subapical region in polarized Müller glia cells

Agnes G.S.H. van Rossum1, Wendy M. Aartsen1, Jan Meuleman1, Jan Klooster2, Anna Malysheva1, Inge Versteeg1, Jean-Pierre Arsanto3, André Le Bivic3 and Jan Wijnholds1,*

1 Department of Neuromedical Genetics, 2 Department of Retinal Signal Processing, The Netherlands Institute for Neuroscience (NIN), Royal Netherlands Academy of Arts and Sciences (KNAW), Meibergdreef 47, 1105 BA Amsterdam, The Netherlands and 3 UMR CNRS 6216, Institute of Developmental Biology of Marseille-Luminy, Faculté des Sciences de Luminy, Case 907, 13288 Marseille Cedex 09, France

* To whom correspondence should be addressed. Tel: +31 205664597; Fax: +31 205666121; Email: j.wijnholds{at}nin.knaw.nl

Received April 26, 2006; Accepted July 26, 2006

Mutations in the human Crumbs homologue-1 (CRB1) gene cause retinal diseases including Leber's congenital amaurosis (LCA) and retinitis pigmentosa type 12. The CRB1 transmembrane protein localizes at a subapical region (SAR) above intercellular adherens junctions between photoreceptor and Müller glia (MG) cells. We demonstrate that the Crb1–/– phenotype, as shown in Crb1–/– mice, is accelerated and intensified in primary retina cultures. Immuno-electron microscopy showed strong Crb1 immunoreactivity at the SAR in MG cells but barely in photoreceptor cells, whereas Crb2, Crb3, Patj, Pals1 and Mupp1 were present in both cell types. Human CRB1, introduced in MG cells in Crb1–/– primary retinas, was targeted to the SAR. RNA interference-induced silencing of the Crb1-interacting-protein Pals1 (protein associated with Lin7; Mpp5) in MG cells resulted in loss of Crb1, Crb2, Mupp1 and Veli3 protein localization and partial loss of Crb3. We conclude that Pals1 is required for correct localization of Crb family members and its interactors at the SAR of polarized MG cells.


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