Publication Date:
1992-04-24
Description:
The gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) is defective in patients with cystic fibrosis. Although the protein product of the CFTR gene has been proposed to function as a chloride ion channel, certain aspects of its function remain unclear. The role of CFTR in the adenosine 3',5'-monophosphate (cAMP)-dependent regulation of plasma membrane recycling was examined. Adenosine 3',5'-monophosphate is known to regulate endocytosis and exocytosis in chloride-secreting epithelial cells that express CFTR. However, mutant epithelial cells derived from a patient with cystic fibrosis exhibited no cAMP-dependent regulation of endocytosis and exocytosis until they were transfected with complementary DNA encoding wild-type CFTR. Thus, CFTR is critical for cAMP-dependent regulation of membrane recycling in epithelial tissues, and this function of CFTR could explain in part the pleiotropic nature of cystic fibrosis.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Bradbury, N A -- Jilling, T -- Berta, G -- Sorscher, E J -- Bridges, R J -- Kirk, K L -- New York, N.Y. -- Science. 1992 Apr 24;256(5056):530-2.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Physiology and Biophysics, University of Alabama, Birmingham 35294.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/1373908" target="_blank"〉PubMed〈/a〉
Keywords:
Base Sequence
;
Cell Membrane/*physiology
;
Chlorides/metabolism
;
Colforsin/pharmacology
;
Cyclic AMP/pharmacology
;
Cystic Fibrosis/*physiopathology
;
Cystic Fibrosis Transmembrane Conductance Regulator
;
DNA/genetics
;
Endocytosis/drug effects/physiology
;
Epithelium/secretion
;
Exocytosis/drug effects/physiology
;
Gene Expression
;
Horseradish Peroxidase/metabolism
;
Humans
;
Membrane Proteins/genetics/*physiology
;
Molecular Sequence Data
;
Pancreatic Neoplasms
;
Transfection
;
Tumor Cells, Cultured
;
Wheat Germ Agglutinins/metabolism
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
