Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth

Author:

Stoltz David A.1,Meyerholz David K.2,Pezzulo Alejandro A.1,Ramachandran Shyam3,Rogan Mark P.1,Davis Greg J.1,Hanfland Robert A.4,Wohlford-Lenane Chris3,Dohrn Cassie L.2,Bartlett Jennifer A.3,Nelson George A.1,Chang Eugene H.5,Taft Peter J.1,Ludwig Paula S.1,Estin Mira1,Hornick Emma E.1,Launspach Janice L.1,Samuel Melissa6,Rokhlina Tatiana1,Karp Philip H.17,Ostedgaard Lynda S.1,Uc Aliye3,Starner Timothy D.3,Horswill Alexander R.8,Brogden Kim A.9,Prather Randall S.6,Richter Sandra S.2,Shilyansky Joel4,McCray Paul B.3,Zabner Joseph1,Welsh Michael J.1710

Affiliation:

1. Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

2. Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

3. Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

4. Department of Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

5. Department of Otolaryngology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

6. Division of Animal Sciences, University of Missouri, Columbia, MO 65211, USA.

7. Howard Hughes Medical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

8. Department of Microbiology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

9. Department of Periodontics and Dows Institute for Dental Research, College of Dentistry, University of Iowa, Iowa City, IA 52242, USA.

10. Department of Molecular Physiology and Biophysics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.

Abstract

The lungs of just-born piglets with cystic fibrosis fail to efficiently eliminate bacteria, suggesting that lung problems in cystic fibrosis patients may be secondary to impaired antibacterial defense mechanisms.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

General Medicine

Reference42 articles.

1. Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA

2. M. J. Welsh B. W. Ramsey F. Accurso G. R. Cutting Cystic fibrosis in The Metabolic and Molecular Basis of Inherited Disease C. R. Scriver A. L. Beaudet W. S. Sly D. Valle B. Childs B. Vogelstein Eds. (McGraw-Hill New York 2001) pp. 5121–5189.

3. Physiological Basis of Cystic Fibrosis: A Historical Perspective

4. Cystic Fibrosis

5. Cystic Fibrosis and the Salt Controversy

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