A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance.

ABSTRACT: Mucociliary clearance, the physiological process by which mammalian conducting airways expel pathogens and unwanted surface materials from the respiratory tract, depends on the coordinated function of multiple specialized cell types, including basal stem cells, mucus-secreting goblet cells, motile ciliated cells, cystic fibrosis transmembrane conductance regulator (CFTR)-rich ionocytes, and immune cells^1,2. Bronchiectasis, a syndrome of pathological airway dilation associated with impaired mucociliary clearance, may occur sporadically or as a consequence of Mendelian inheritance, for example in cystic fibrosis, primary ciliary dyskinesia (PCD), and select immunodeficiencies³. Previous studies have identified mutations that affect ciliary structure and nucleation in PC

SUBMITTER: Chivukula RR 

PROVIDER: S-EPMC7018620 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature