National Academy of Sciences, Proceedings of the National Academy of Sciences, 26(114), p. 6842-6847, 2017
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Significance Mucus propelled by cilia is key for removing particulates from lungs by mucociliary transport. The major structural components of airway mucus are two gel-forming mucins, MUC5B and MUC5AC. These mucins exhibit distinct morphologic structures. MUC5B is secreted by submucosal glands in the form of strands. MUC5AC is secreted by goblet cells as threads and thin sheets. After emerging onto the airway surface, the two mucins associate to form MUC5B strands partially covered with MUC5AC. These distinct morphologic structures likely enable efficient mucociliary transport. In cystic fibrosis, strands become entangled, MUC5B often fills submucosal gland ducts, and MUC5AC sheets are larger, are more abundant, and overlie strands. Disrupted anion secretion in cystic fibrosis alters mucin morphology, which will impair mucociliary transport.