Cellular signalling by primary cilia in development, organ function and disease
TLDR
This Review describes the main signalling pathways that are coordinated by primary cilia to control developmental processes, tissue plasticity and organ function and how defects in the output of ciliary signalling events are coupled to developmental disorders and disease progression.Abstract:
Primary cilia project in a single copy from the surface of most vertebrate cell types; they detect and transmit extracellular cues to regulate diverse cellular processes during development and to maintain tissue homeostasis. The sensory capacity of primary cilia relies on the coordinated trafficking and temporal localization of specific receptors and associated signal transduction modules in the cilium. The canonical Hedgehog (HH) pathway, for example, is a bona fide ciliary signalling system that regulates cell fate and self-renewal in development and tissue homeostasis. Specific receptors and associated signal transduction proteins can also localize to primary cilia in a cell type-dependent manner; available evidence suggests that the ciliary constellation of these proteins can temporally change to allow the cell to adapt to specific developmental and homeostatic cues. Consistent with important roles for primary cilia in signalling, mutations that lead to their dysfunction underlie a pleiotropic group of diseases and syndromic disorders termed ciliopathies, which affect many different tissues and organs of the body. In this Review, we highlight central mechanisms by which primary cilia coordinate HH, G protein-coupled receptor, WNT, receptor tyrosine kinase and transforming growth factor-β (TGFβ)/bone morphogenetic protein (BMP) signalling and illustrate how defects in the balanced output of ciliary signalling events are coupled to developmental disorders and disease progression.read more
Citations
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Autophagy in kidney homeostasis and disease.
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The Hippo Pathway, YAP/TAZ and the Plasma Membrane
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Pathophysiological implications of hypoxia in human diseases
Pai Sheng Chen,Wen Tai Chiu,Pei Ling Hsu,Shih Chieh Lin,I-Chen Peng,Chia Yih Wang,Shaw Jenq Tsai +6 more
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The molecular structure of mammalian primary cilia revealed by cryo-electron tomography.
Petra Kiesel,Gonzalo Alvarez Viar,Nikolai Tsoy,Riccardo Maraspini,Peter Gorilak,Vladimir Varga,Alf Honigmann,Gaia Pigino +7 more
TL;DR: This work developed an enabling method for investigating the structure of primary cilia isolated from MDCK-II cells at molecular resolution by cryo-electron tomography and shows that the textbook '9 + 0' arrangement of microtubule doublets is only present at the primary cilium base.
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First contact: the role of respiratory cilia in host-pathogen interactions in the airways.
Li Eon Kuek,Robert J. Lee +1 more
TL;DR: The expression, structure, and function of respiratory cilia during pathogenic infection of the airways is examined and specific known points of interaction of bacteria, fungi, and viruses with respiratory cili function are discussed.
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