Proceedings of the National Academy of Sciences · 1990 · 716 citations · 39 references
Life SciencesBiotechnologyMolecular BiologyClinical BiochemistryMolecular BiotechnologyRecombinant Dna TechnologyCystic FibrosisCystic Fibrosis PharmacotherapyCystic Fibrosis Lung DiseaseInhalable Biological TherapeuticsViscous Effects
Respiratory distress and progressive lung destruction in cystic fibrosis can be attributed to bacterial persistence and the accumulation of viscous purulent secretions in the airways. More than 30 yr ago it was suggested that the large amounts of DNA in purulent secretions contribute to its viscosity and that bovine pancreatic DNase I could reduce the viscosity. To evaluate the potential clinical utility of recombinant human DNase I (rhDNase) in the treatment of cystic fibrosis, we have cloned, sequenced, and expressed rhDNase. Catalytic amounts of rhDNase greatly reduce the viscosity of purulent cystic fibrosis sputum, transforming it within minutes from a nonflowing viscous gel to a flowing liquid. The reduction in viscosity is associated with a decrease in size of DNA in the sputum. Inhalation of a rhDNase aerosol may be a simple direct approach that will help individuals with cystic fibrosis and other patients with pneumonia or bronchitis to clear their airways of purulent secretions.
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Identification of the Cystic Fibrosis Gene: Genetic Analysis
Bat-Sheva Kerem, Johanna M. Rommens, Janet A. Buchanan et al. · Science · 1989 · 4.1K citations
Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
Johanna M. Rommens, Michael C. Iannuzzi, Bat-Sheva Kerem et al. · Science · 1989 · 3.2K citations
Lutz Nährlich, Jochen G. Mainz, Constantin Adams et al. · Cellular Physiology and Biochemistry · 2013 · 2.5K citations · Full text