Composition and function of the Crumbs protein complex in the mammalian retina

Experimental Eye Research
Ilse GosensRonald Roepman

Abstract

The Crumbs proteins (CRBs) are transmembrane proteins, homologous to Drosophila Crumbs, with a key role in defining the apical membrane domain in photoreceptors as well as in embryonic epithelia. Crumbs proteins are conserved between species and their intracellular domains are involved in organizing a conserved macromolecular protein scaffold with important roles in cell polarity as well as morphogenesis and maintenance of the retina. Mutations in the gene encoding human CRB1, the first one identified out of the three human orthologs, have been associated with a number of retinal dystrophies including Leber amaurosis and retinitis pigmentosa type 12. Although no other mammalian Crumbs complex members as of yet have been associated with retinal degeneration, disruption of different zebrafish and fruitfly orthologs can lead to various retinal defects. The core Crumbs complex localizes apical to the outer limiting membrane, where photoreceptors and Müller glia contact each other. Correct functioning of Crumbs ensures adhesion between these cells by an unknown mechanism. This review summarizes the current view on the composition and function of the Crumbs prsotein complex in the mammalian retina. Recently, a number of new members o...Continue Reading

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