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Studying Ciliary Signaling in Development and Disease

Project description

Characterising regulation of cellular 'hairs' could impact numerous heritable conditions

Eukaryotic cilia are small hair-like organelles. Among the best known are the cilia on epithelial cells lining the airways that move mucous up and out. However, cilia can be motile or non-motile, and are a component of almost all vertebrate cells. Ciliopathies are a rapidly emerging group of human disorders that affect nearly all organ systems. They are associated with inherited mutations, with the number of identified associated genes rising from over 40 genes in 2011 to over 150 by 2017. Prevalent phenotypes include polycystic kidney disease, retinal degeneration, obesity, skeletal malformations and brain anomalies. SCilS is training a new generation of scientists to characterise this increasingly important and diverse class of inherited diseases.

Objective

SCilS will create a multidisciplinary and intersectoral European training network focusing on ciliary signalling in development and disease.
Primary cilia are microtubule-based cell surface projections that have evolved to be key signalling hubs of our cells, as they concentrate or segregate components of major cellular signalling pathways. Control of ciliary signaling output requires a high degree of regulation and critical feedback, which is needed for robustness in development and cellular homeostasis of different tissues and organs. Dysfunctional cilia can therefore lead to >35 severe human genetic traits (ciliopathies) with highly heterogeneous, overlapping phenotypes. Ciliopathies affect as many as 1 in 400 people, and for the majority of cases efficient therapeutic interventions are currently unavailable.
SCilS research aims to uncover the multi-level organization and regulation of cilia-mediated signalling pathways in order to understand ciliopathy disease etiology and identify novel therapeutic targets. This challenging task will be accomplished by integrating unique expertise and cutting edge technology available within the SCilS network, including structural biology, super resolution imaging and cryo-electron tomography, state-of-the-art genomics, proteomics and bioinformatics, (stem) cell biology and biochemistry, as well as organoid technology and zebrafish models.
SCilS training will give Early Stage Researchers (ESRs) unparalleled training opportunities in outstanding academic and industrial settings through training-by-research via individual research projects, secondments, and network-wide training sessions. All individual training and research activities have been designed to provide each ESR with the necessary broad competences in state‐of‐the art academic and industrial research. The network will thereby make a career in both industry and academia attractive to the ESRs and improve their career prospects in both private and public sectors.

Coordinator

STICHTING RADBOUD UNIVERSITAIR MEDISCH CENTRUM
Net EU contribution
€ 531 239,76
Address
GEERT GROOTEPLEIN 10 ZUID
6525 GA Nijmegen
Netherlands

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Region
Oost-Nederland Gelderland Arnhem/Nijmegen
Activity type
Higher or Secondary Education Establishments
Links
Total cost
€ 531 239,76

Participants (10)