Proceedings of the National Academy of Sciences · 1990 · 711 citations · 39 references
Dna AnalysisMolecular BiologyNucleic Acid Amplification TestGene DeliveryRecombinant Human DnaseMolecular DiagnosticsCystic Fibrosis SputumOligonucleotideDna ReplicationProgressive Lung DestructionCell BiologyClinical MicrobiologyRhdnase AerosolNatural SciencesBiotechnologyNucleic Acid AmplificationProtein EngineeringMicrobiologyMedicineConnective Tissue Disease
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: Cloning and Characterization of Complementary DNA
John R. Riordan, Johanna M. Rommens, Bat-Sheva Kerem et al. · Science · 1989 · 7.4K citations
Identification of the Cystic Fibrosis Gene: Genetic Analysis
Bat-Sheva Kerem, Johanna M. Rommens, Janet A. Buchanan et al. · Science · 1989 · 4.1K citations
Cystic Fibrosis Gene, Mendelian Disorder, Phenylalanine Residue +12
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