First published online August 17, 2004
Journal of Cell Science 117, 1801e (2004)
© The Company of Biologists Limited
Crumbs of retinal comfort
A major cause of childhood blindness is Leber congenital amaurosis, a group of severe inherited retinal diseases characterised by loss of vision within a year of birth. Mutations in CRB1, the human homologue of the Drosophila junctional protein Crumbs, account for about 10% of these cases and are also implicated in progressive retinitis pigmentosa. To investigate the role of mammalian Crb1 in retinal dystrophies, Jan Wijnholds and colleagues have generated and characterised Crb1-/- mice (see p. 4169). When exposed to normal light-dark cycles, these animals initially have normal retinas, but at 3-9 months old delamination of the photoreceptor layer of their retinas in localised regions begins and is followed by death of retinal neurons. Early exposure to constant light increases the number of lesions. The authors show that disease onset is due to the transient loss of cell adhesion between photoreceptor cells and Müller glial cells, and propose that Crb1 is needed during light exposure to maintain adherens junctions between these two cell types and thus prevent retinal disorganisation and dystrophy.
Related articles in JCS:
- Crumbs homologue 1 is required for maintenance of photoreceptor cell polarization and adhesion during light exposure
- Serge A. van de Pavert, Albena Kantardzhieva, Anna Malysheva, Jan Meuleman, Inge Versteeg, Christiaan Levelt, Jan Klooster, Sylvia Geiger, Mathias W. Seeliger, Penny Rashbass, Andre Le Bivic, and Jan Wijnholds
JCS 2004 117: 4169-4177.
[Abstract]
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