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Role of Crumbs 3 and its partners in vesicular transport and ciliogenesis

Project information

Grant agreement ID: 46575

  • Start date

    1 February 2007

  • End date

    31 January 2009

Funded under:

FP6-MOBILITY

Coordinated by:

CENTRE NATIONAL POUR LA RECHERCHE SCIENTIFIQUE

France

Objective

One person in 3000 in the world suffers from Retinis Pigmentosa, which leads to degeneration of essential cells of retina: the polarized photosensitive cells or photoreceptors. Several forms of Retinis Pigmentosa can cause partial or total blindness.

Their origin is still unknown and there is no efficient therapy to cure or even prevent them. Numerous studies have shown that CRB1, one of the 3 genes encoding for the human family of proteins CRUMBS (Crb1, 2 and 3), is mutated in 60% of patients suffering from a severe form of autosomal recessive Retinis Pigmentosa called RP12. Other reports implicate Crb3 in vesicular transport at the apical membrane of epithelial polarized cells and at the tight junctions between those cells. The function and regulation of the Crumbs family of proteins are unknown.

However, it has been shown that Crb2 and Crb3 are necessary for the formation of the primary cilium at the apical membrane of epithelial cells. The physiological role of the primary cilium remains elusive. Interestingly, several proteins that are localized to the primary cilium are responsible, when mutated, for diseases like the Bardet-Biedl syndrome, polycystic kidney disease or Retinis Pigmentosa. The aim of this project is to study the cellular and molecular role of the Crumbs protein family in morphogenesis and physiology of the retina.

We want to understand the mechanisms by which the Crumbs proteins are necessary for cell polarity establishment by the formation and the maintenance of the apical membrane, the tight junctions, as well as the primary cilium. More precisely, we will seek for the role of Crb3 and its partners in the apical membrane morphogenesis and physiology, in epithelial cells in general, and in photoreceptors in particular.

The results of this research should allow the opening of new avenues for molecular and genetic studies on human retina degeneration and lead to new strategies for therapy against Retinis Pigmentosa and other degeneration diseases.

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Coordinator

CENTRE NATIONAL POUR LA RECHERCHE SCIENTIFIQUE

Address

Rue Michel-Ange, 3
Paris

France

Project information

Grant agreement ID: 46575

  • Start date

    1 February 2007

  • End date

    31 January 2009

Funded under:

FP6-MOBILITY

Coordinated by:

CENTRE NATIONAL POUR LA RECHERCHE SCIENTIFIQUE

France