Cilia
Displaying results 1-22 of 22
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Polycystins may regulate a cilia-dependent signal in kidney tubule cells but are mutated in autosomal dominant polycystic kidney disease (ADPKD). Efforts to unravel the role of cilia and ciliary signaling in ADPKD are ongoing.
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Specialized sensory cilia in the photoreceptors of the eye convert light stimuli into neurological responses. Mutations in various photoreceptor-specific and common cilia genes lead to inherited retinal degenerations.
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Visceral organs in vertebrates are left–right asymmetric with regard to position and morphology. Recent work has advanced our understanding how cilia-driven fluid flow establishes this left–right asymmetry in mouse embryos.
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Some G-protein-coupled receptors are enriched on cilia of certain mammalian cell types (e.g., odorant receptors in olfactory sensory neuron cilia). The significance of this location specificity is beginning to be understood.
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The craniofacial complex is the main organ system affected in
30% of ciliopathies. This may be because craniofacial development involves cells and signaling pathways (e.g., Hedgehog) that
heavily depend on proper ciliary function.
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Unlike most cells, the axonemes of many sperm tails extend into the cytoplasm. This may be caused by the migration of the transition zone (at the axoneme–centriole junction) away from its typical position at the cell membrane.
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Congenital heart disease (CHD) patients often exhibit ciliary dysfunction, and studies in mice have revealed a central role for cilia in CHD pathogenesis. CHD may therefore represent a new class of ciliopathy.
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Cell signaling machinery involved in feeding behavior and energy homeostasis localizes to certain neuronal cilia. This may help explain why ciliary dysfunction can lead to obesity (e.g., in Bardet–Biedl Syndrome).
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Various chemical and physical cues guide sperm to eggs, depending on the species. In general, these cues activate signaling pathways that evoke a cellular Ca2+ response and modulate the waveform of the flagellar beat.
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Posttranslational modifications accumulate on certain microtubules in cilia (e.g., the B-tubules of the axoneme) but not on others (e.g., the A-tubules). These and other nonuniform patterns may have functional consequences.
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Primary cilia help coordinate a cell's response to changes in the extracellular environment. In mammals, the RTK and TGF-β pathways are involved. Defects in these pathways are linked to ciliopathies.
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Radial spokes are multiprotein structures in the axonemes of most cilia that are critical for motility control. Recent studies have made progress in characterizing their structure, molecular interactions, and evolution.
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Hedgehog signaling is essential for the development and maintenance of most organs. In vertebrates, Hedgehog signaling completely depends on a highly specialized organelle: the primary cilium.
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Ciliated cells in the lungs play a key role in mucociliary clearance, propelling inhaled particles out of the airways. Chronic diseases of the airways (e.g., primary ciliary dyskinesia) have offered insight into this process.
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Nephronophthisis and nephronophthisis-related ciliopathies are inherited disorders caused by mutations in genes involved in ciliary function. They are genetically and phenotypically very heterogeneous.
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Intriguing insights into the assembly, composition, and function of the central microtubules and associated proteins in motile cilia have recently been made. For example, katanin—a protein that severs microtubules—has been implicated in their assembly.
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The ciliary gate is the region at the base of the cilium that separates it from the rest of the cell. The gate consists of transition fibers and a transition zone that regulate proteins entering and exiting the cilium.
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Motile cilia are cell-surface structures that move (e.g., to sweep dirt from the lungs). Recent structural studies have provided insight into the components, dynamics, and evolution of the axoneme—the main internal structure.
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The last eukaryotic common ancestor was remarkably complex, possessing motile cilia built on a typical 9+2 microtubule array and capable of gliding motility, beating motility, and display of sensory receptors.
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Multiciliated cells (e.g., those in human airways) possess up to 300 motile cilia that beat in a coordinated fashion. Over the past decade, remarkable progress has been made in understanding how these cells develop and function.
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In motile cilia, movement is ultimately generated by dynein arms associated with axonemal microtubules. These dynein arms are complex structures that are assembled in the cytoplasm and trafficked into the ciliary shaft.
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Between the base and the tip of each cilium is a multicomponent, bidirectional transport system. Recent work has provided insights into its molecular structure and mechanisms (e.g., in cargo recognition and transport).
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