6533b830fe1ef96bd1297d15
RESEARCH PRODUCT
SYSCILIA, “A systems biology approach to dissect cilia function and its disruption in human genetic disease”
M PontoglioNicholas KatsanisOliver E. BlacqueMarius UeffingToby J. GibsonRobert B. RussellFrançois KépèsRonald RoepmanColin A. JohnsonG ApicGt WalzMartijn A. HuynenHeymut OmranPl BealesHannie KremerRachel H. GilesUwe WolfrumBrunella Francosubject
Systems biologyCiliumProteomePoster PresentationWnt signaling pathwayCell BiologyComputational biologyBiologyProteomicsDevelopmental biologyHedgehogHuman geneticsCell biologydescription
Primary cilia are basically signaling hubs, harboring amongst others the noncanonical WNT, Hedgehog,and PDGF signaling systems, and their disruption leads to striking developmental defects. Some ciliopathy-associated proteins have recently been revealed to be physically or functionally associated in several distinct groupings, with limited connections to other crucial biological processes. Early proteomics studies have also suggested a discrete repertoire of about 1000 proteins within the organelle (i.e. <5% of the proteome) that are still in need of organisation into pathways and networks. Small, relatively isolated systems are often targeted by systems biology approaches under the assumption that a limited set of molecules and interactions will be more tractable for modelling systems. Cilia are thus ideal organelles for systems biology as they can be regarded as semi-closed systems being both largely spatially and biologically separated from many other cellular structures and processes.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2012-11-01 | Cilia |