SYSCILIA
A systems biology approach to dissect cilia function and its disruption in human genetic disease
| Coordinatore | STICHTING KATHOLIEKE UNIVERSITEIT
Organization address
address: GEERT GROOTEPLEIN NOORD 9 contact info |
| Nazionalità Coordinatore | Netherlands [NL] |
| Totale costo | 14˙939˙311 € |
| EC contributo | 11˙069˙083 € |
| Programma | FP7-HEALTH
Specific Programme "Cooperation": Health |
| Code Call | FP7-HEALTH-2009-two-stage |
| Funding Scheme | CP-IP |
| Anno di inizio | 2010 |
| Periodo (anno-mese-giorno) | 2010-06-01 - 2015-05-31 |
Partecipanti
| # | ||||
|---|---|---|---|---|
| 1 |
STICHTING KATHOLIEKE UNIVERSITEIT
Organization address
address: GEERT GROOTEPLEIN NOORD 9 contact info |
NL (NIJMEGEN) | coordinator | 2˙120˙978.00 |
| 2 |
EBERHARD KARLS UNIVERSITAET TUEBINGEN
Organization address
address: GESCHWISTER-SCHOLL-PLATZ contact info |
DE (TUEBINGEN) | participant | 1˙230˙600.00 |
| 3 |
DUKE UNIVERSITY
Organization address
address: BLACKWELL ST SUITE 920 324 contact info |
US (DURHAM) | participant | 888˙000.00 |
| 4 |
UNIVERSITY OF LEEDS
Organization address
address: WOODHOUSE LANE contact info |
UK (LEEDS) | participant | 722˙500.00 |
| 5 |
UNIVERSITAIR MEDISCH CENTRUM UTRECHT
Organization address
address: HEIDELBERGLAAN 100 contact info |
NL (UTRECHT) | participant | 705˙000.00 |
| 6 |
FONDAZIONE TELETHON
Organization address
address: VIA VARESE 16/B contact info |
IT (ROMA) | participant | 684˙975.00 |
| 7 |
JOHANNES GUTENBERG UNIVERSITAET MAINZ
Organization address
address: SAARSTRASSE 21 contact info |
DE (MAINZ) | participant | 639˙800.00 |
| 8 |
UNIVERSITY COLLEGE LONDON
Organization address
address: GOWER STREET contact info |
UK (LONDON) | participant | 639˙400.00 |
| 9 |
Cambridge Cell Networks Ltd
Organization address
address: St Johns Innovation Centre contact info |
UK (Cambridge) | participant | 578˙400.00 |
| 10 |
UNIVERSITY COLLEGE DUBLIN, NATIONAL UNIVERSITY OF IRELAND, DUBLIN
Organization address
address: BELFIELD contact info |
IE (DUBLIN) | participant | 531˙600.00 |
| 11 |
UNIVERSITAETSKLINIKUM FREIBURG
Organization address
address: HUGSTETTER STRASSE 49 contact info |
DE (FREIBURG) | participant | 422˙400.00 |
| 12 |
WESTFAELISCHE WILHELMS-UNIVERSITAET MUENSTER
Organization address
address: SCHLOSSPLATZ 2 contact info |
DE (MUENSTER) | participant | 422˙400.00 |
| 13 |
INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)
Organization address
address: 101 Rue de Tolbiac contact info |
FR (PARIS) | participant | 411˙800.00 |
| 14 |
RUPRECHT-KARLS-UNIVERSITAET HEIDELBERG
Organization address
address: SEMINARSTRASSE 2 contact info |
DE (HEIDELBERG) | participant | 360˙550.00 |
| 15 |
EUROPEAN MOLECULAR BIOLOGY LABORATORY
Organization address
address: Meyerhofstrasse 1 contact info |
DE (HEIDELBERG) | participant | 350˙080.00 |
| 16 |
UNIVERSITE D'EVRY-VAL D'ESSONNE
Organization address
address: BOULEVARD FRANCOIS MITTERAND 23 1 contact info |
FR (EVRY) | participant | 200˙084.40 |
| 17 |
CENTRE NATIONAL DE LA RECHERCHE SCIENTIFIQUE
Organization address
address: Rue Michel -Ange 3 contact info |
FR (PARIS) | participant | 160˙515.60 |
Mappa
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Obiettivo del progetto (Objective)
'AIM: To identify the molecular mechanisms characterizing cilium function, and the discrete perturbations associated with dysfunction caused by mutations in inherited ciliopathies, applying a systems biology approach. BACKGROUND: Cilia are microtubule-based, centriole-derived projections from the cell surface. They transduce extracellular signals and regulate key processes in which signals of the extracellular environment are translated into a cellular response, such as cell cycle control, Wnt signalling, Shh signalling and planar cell polarity. Disruption of cilium-based processes by mutations can cause very severe disorders. Many of these ciliopathies have overlapping phenotypes. There is evidence, that ciliary proteins are organized in cell/context specific complexes and/or in shared regulatory circuits in cilia of affected tissues. Yet, knowledge of the composition, wiring, dynamics and associated signaling pathways of the corresponding molecular building blocks and associated protein networks remains very limited. APPROACH: We propose here that ciliopathies can be considered systemically as specific perturbations in a versatile dynamically regulated multifunctional molecular machine. Mainly based on the comprehensive description of the ciliary interactome, quantitative functional assays as well as human genetic data derived from ciliopathy patients, we will generate a comprehensive stream of content-rich quantitative data towards systemic analysis of ciliar function. These data will be used to generate and validate discrete models that describe functional modules and regulatory circuits in the ciliome as well as predicting biological context specific features of cilia as well as perturbations leading to ciliopathies. This will enable us to 1) understand the systemic features of discrete ciliary functions, 2) scrutinize the molecular disease mechanisms of different overlapping ciliopathies, and 3) develop therapeutic strategies towards improved treatment.'
Introduzione (Teaser)
The advent of molecular analysis technologies has facilitated the identification of novel protein networks as therapeutic targets. Combining systems biology and animal modelling, a European study aimed to reach beyond the state of the art in the field of cilia function and disease, and discover novel therapies.
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