Journal article
A Controlled Study of Adenoviral-Vector–Mediated Gene Transfer in the Nasal Epithelium of Patients with Cystic Fibrosis
The New England journal of medicine, Vol.333(13), pp.823-831
09/28/1995
DOI: 10.1056/NEJM199509283331302
PMID: 7544439
Abstract
Cystic fibrosis is a recessive genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (
CFTR
) gene.
1
–
4
The normal
CFTR
gene codes for a protein (CFTR) that plays a key part in epithelial transport of salt and water.
5
,
6
Mutations in
CFTR
result in abnormal secretions that obstruct and ultimately damage epithelium in many areas of the body.
7
The principal cause of death among patients with cystic fibrosis is lung disease. Patients who are homozygous for mutations in the
CFTR
gene have defective cyclic AMP (cAMP)–regulated secretion of chloride
8
,
9
and elevated absorption of sodium . . .
Details
- Title: Subtitle
- A Controlled Study of Adenoviral-Vector–Mediated Gene Transfer in the Nasal Epithelium of Patients with Cystic Fibrosis
- Creators
- Michael R Knowles - Cystic Fibrosis–Pulmonary Research and Treatment CenterKathy W Hohneker - Cystic Fibrosis–Pulmonary Research and Treatment CenterZhaoqing Zhou - Cystic Fibrosis–Pulmonary Research and Treatment CenterJohn C Olsen - Cystic Fibrosis–Pulmonary Research and Treatment CenterTerry L Noah - Departments of PediatricsPing-Chuan Hu - University of North CarolinaMargaret W Leigh - Departments of PediatricsJohn F Engelhardt - University of North CarolinaLloyd J EdwardsKim R Jones - Cystic Fibrosis–Pulmonary Research and Treatment CenterMariann Grossman - University of North CarolinaJames M Wilson - University of North CarolinaLarry G Johnson - Cystic Fibrosis–Pulmonary Research and Treatment CenterRichard C Boucher - Cystic Fibrosis–Pulmonary Research and Treatment Center
- Resource Type
- Journal article
- Publication Details
- The New England journal of medicine, Vol.333(13), pp.823-831
- DOI
- 10.1056/NEJM199509283331302
- PMID
- 7544439
- NLM abbreviation
- N Engl J Med
- ISSN
- 0028-4793
- eISSN
- 1533-4406
- Publisher
- Massachusetts Medical Society
- Language
- English
- Date published
- 09/28/1995
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Anatomy and Cell Biology; Radiation Oncology; Internal Medicine
- Record Identifier
- 9984284352802771
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