A Pilot Clinical Trial of Oral Sodium 4-Phenylbutyrate (Buphenyl) in Δ F508-Homozygous Cystic Fibrosis Patients
Author:
Publisher
American Thoracic Society
Subject
Critical Care and Intensive Care Medicine,Pulmonary and Respiratory Medicine
Reference28 articles.
1. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
2. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
3. Degradation of CFTR by the ubiquitin-proteasome pathway
4. The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR
5. Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
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