Cystic Fibrosis

A breathtaking tale of sticky mucus

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Science  15 Aug 2014:
Vol. 345, Issue 6198, pp. 782
DOI: 10.1126/science.345.6198.782-f

Patients with cystic fibrosis have difficulty breathing because their airways are clogged with thick mucus. Does this mucus accumulate because there is a defect in the way it is produced? Or does it accumulate because of other disease features, such as dehydration or airway wall remodeling? Distinguishing between these possibilities is important for future drug development. In a study of piglets with cystic fibrosis, Hoegger et al. identify mucus production as the primary defect (see the Perspective by Wine). The airway glands of the piglets synthesized strands of mucus normally, but the strands were never released and stayed tethered to the gland ducts.

Science, this issue p. 818; see also p. 730

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