Related Experiment Video
Updated: Jun 24, 2026

In vivo Evaluation of Mucociliary Clearance in Mice
Published on: December 18, 2020
Inactivation of Chibby affects function of motile airway cilia
Vera A Voronina1, Ken-Ichi Takemaru, Piper Treuting
1Department of Pharmacology, Howard Hughes Medical Institute, USA.
Abstract:
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 with Pseudomonas aeruginosa isolates, Cby(-/-) mice are unable to clear the bacteria from the nasal cavity. Notably, Cby(-/-) mice exhibit a complete absence of mucociliary transport caused by a marked paucity of motile cilia in the nasal epithelium. Moreover, ultrastructural experiments reveal impaired basal body docking to the apical surface of multiciliated cells. In support of these phenotypes, endogenous Cby protein is localized at the base of cilia. As the phenotypes of Cby(-/-) mice bear striking similarities to primary ciliary dyskinesia, Cby(-/-) mice may prove to be a useful model for this condition.
Related Concept Videos
Mechanism of Ciliary Motion
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
Mechanism of Ciliary Motion
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
GPCRs Regulate Adenylyl Cylase Activity
Two...
Cholinergic Antagonists: Pharmacological Actions
Gastrointestinal Effects: Antimuscarinics reduce gut contractions, increase gastric emptying, and slow intestinal transit. They partly inhibit gastric acid secretion...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Indirect-Acting Cholinergic Agonists: Mechanism of Action
Reversible inhibitors like edrophonium bind to a specific part of the enzyme called the anionic catalytic site. They form noncovalent bonds, which means they are not strongly attached to the enzyme. This creates a temporary and less stable enzyme–inhibitor complex, leading to...

