Motile and non-motile cilia in human pathology: from function to phenotypes

Structural maintenance and chemosensory function of human airway motile cilia

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SnapShot: Motile Cilia

Inactivation of Chibby affects function of motile airway cilia

Chibby (Cby) is a conserved component of the Wnt-beta-catenin pathway. Cby physically interacts with beta-catenin to repress its activation of transcription. To elucidate the function of Cby in vertebrates, we generated Cby(-/-) mice and found that after 2-3 d of weight loss, the majority of mice die before or around weaning. All Cby(-/-) mice develop rhinitis and sinusitis. When challenged wit...

Non-motile primary cilia as fluid shear stress mechanosensors.

Primary cilia are sensory organelles that transmit extracellular signals into intracellular biochemical responses. Structural and functional defects in primary cilia are associated with a group of human diseases, known as ciliopathies, with phenotypes ranging from cystic kidney and obesity to blindness and mental retardation. Primary cilia mediate mechano- and chemosensation in many cell types....

Motile cilia-specific protein macrocomplexes and cilia beating

In Eukaryotes, except fungi and higher plants, the coordinated beating of the motile cilia propels unicellular organisms and regulate the flow of the extracellular fluids in multicellular life forms. In humans proper beating of these tiny nanomachines ensures removal of the mucus and bacteria out of the airways, circulation of cerebrospinal fluid in brain ventricles, transport of the oocytes in...