Journal Article

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

Agnes G.S.H. van Rossum, Wendy M. Aartsen, Jan Meuleman, Jan Klooster, Anna Malysheva, Inge Versteeg, Jean-Pierre Arsanto, André Le Bivic and Jan Wijnholds

in Human Molecular Genetics

Volume 15, issue 18, pages 2659-2672
Published in print September 2006 | ISSN: 0964-6906
Published online August 2006 | e-ISSN: 1460-2083 | DOI: http://dx.doi.org/10.1093/hmg/ddl194
Pals1/Mpp5 is required for correct localization of Crb1 at the subapical region in polarized Müller glia cells

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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.

Journal Article.  7921 words.  Illustrated.

Subjects: Genetics and Genomics

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