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CilMitoPatho Report Summary

Project ID: 333959
Funded under: FP7-PEOPLE
Country: France

Final Report Summary - CILMITOPATHO (Investigating the role of cilia proteins in dividing cells: implications in kidney cyst formation and ciliopathies.)

Polycystic kidney disease is characterized by kidney tubules and lumen disorganization. It has long been associated exclusively with dysfunctions of cilia, sensory organelles required for proper cellular functions within tissues. However, recent results, including ours, indicate that intracellular transport complexes involved in kidney cyst formation and initially described for their ciliary role, also have non-ciliary functions that may contribute to the development of the disease.

The overall objective of our project has been to investigate non-ciliary functions of cilia proteins and to tackle from a different angle their implication in pathologies. More specifically, we were interested in understanding how proteins of the intraflagellar transport machinery (IFT), well characterized for their ciliary functions, regulate cell division. Using cutting edge biochemical and microscopy techniques, we thus characterized at the cellular and molecular levels the role of the IFT machinery in dividing cells. We also investigated how mitotic dysfunctions induced by IFT proteins depletion can contribute to defects in kidney tissue morphogenesis and to the appearance of kidney cysts, one of the most common ciliopathy-related phenotypes. To achieve this goal we implemented at the CRBM 3D cultures of kidney cells and zebrafish as an in vivo model organism to study cellular processes associated with kidney cyst formation.

Overall, our work has unraveled novel non-ciliary roles for cilia proteins during cell division and suggests that division defects could contribute to kidney cyst formation. It is thus setting the stage for future work in the group that will aim at understanding how mitotic defects contribute to abnormal kidney tissue morphogenesis, kidney cyst formation and cancer. Overall, our future work will aim at getting an integrated view of the cellular mechanisms involved in renal tubule morphogenesis and should shed light on their contribution to pathologies including kidney cyst formation and cancer.

Importantly, by setting up the zebrafish facility at the CRBM we have been asked by non-zebrafish groups to collaborate in order to complement their works with an in vivo approach. Indeed, the rapid development and transparency of the zebrafish embryos allow easy and detailed observations of cellular processes. It thus makes it an ideal platform to study cell biology in a living organism. Zebrafish embryos are also highly suitable for drug testing because they show high permeability for small-molecules. These collaborations successfully led to publications.


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