Skip Navigation



Human Molecular Genetics Advance Access published online on August 2, 2006
Human Molecular Genetics, doi:10.1093/hmg/ddl194
This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow Supplementary Data
Right arrow All Versions of this Article:
15/18/2659    most recent
ddl194v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by van Rossum, A. G.S.H.
Right arrow Articles by Wijnholds, J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by van Rossum, A. G.S.H.
Right arrow Articles by Wijnholds, J.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author 2006. Published by Oxford University Press. All rights reserved
Received April 26, 2006
Revised July 26, 2006
Accepted July 26, 2006

Article

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

Agnes G.S.H. van Rossum 1, Wendy M. Aartsen 1, Jan Meuleman 1, Jan Klooster 2, Anna Malysheva 1, Inge Versteeg 1, Jean-Pierre Arsanto 3, André Le Bivic 3, and Jan Wijnholds 1 *

1 Department of Neuromedical Genetics, The Netherlands Institute for Neuroscience (NIN), Royal Netherlands Academy of Arts and Sciences (KNAW), Meibergdreef 47, 1105 BA Amsterdam, The Netherlands
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
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.
Jan Wijnholds, E-mail: j.wijnholds{at}nin.knaw.nl


  Abstract

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 sub-apical 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. RNAi 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.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 J. Cell Sci.Home page
N. A. Bulgakova and E. Knust
The Crumbs complex: from epithelial-cell polarity to retinal degeneration
J. Cell Sci., August 1, 2009; 122(15): 2587 - 2596.
[Abstract] [Full Text] [PDF]

Home page
 GeneticsHome page
S. Berger, N. A. Bulgakova, F. Grawe, K. Johnson, and E. Knust
Unraveling the Genetic Complexity of Drosophila stardust During Photoreceptor Morphogenesis and Prevention of Light-Induced Degeneration
Genetics, August 1, 2007; 176(4): 2189 - 2200.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
S. A. van de Pavert, J. Meuleman, A. Malysheva, W. M. Aartsen, I. Versteeg, F. Tonagel, W. Kamphuis, C. J. McCabe, M. W. Seeliger, and J. Wijnholds
A Single Amino Acid Substitution (Cys249Trp) in Crb1 Causes Retinal Degeneration and Deregulates Expression of Pituitary Tumor Transforming Gene Pttg1
J. Neurosci., January 17, 2007; 27(3): 564 - 573.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.