Cystic fibrosis gene expression is not correlated with rectifying Cl sup minus channels

• Published in: Proceedings of the National Academy of Sciences - PNAS Vol. 88:12
• Main Authors: Ward, C.L., Krouse, M.E., Kopito, R.R., Wine, J.J., Gruenert, D.C.
• Format: Journal Article
• Language: English
• Published: United States 15.06.1991
• Subjects: 550200 - Biochemistry; ANIMAL TISSUES; ANIMALS; BASIC BIOLOGICAL SCIENCES; BODY; CHLORIDES; CHLORINE COMPOUNDS; DISEASES; DNA POLYMERASES; ENZYMES; EPITHELIUM; ETIOLOGY; FIBROSIS; GENE REGULATION; HALIDES; HALOGEN COMPOUNDS; HEREDITARY DISEASES; MAMMALS; MAN; MEMBRANE TRANSPORT; MESSENGER-RNA; NUCLEIC ACIDS; NUCLEOTIDYLTRANSFERASES; ORGANIC COMPOUNDS; PATHOLOGICAL CHANGES; PERMEABILITY; PHOSPHORUS-GROUP TRANSFERASES; POLYMERASES; PRIMATES; PROTEINS; RNA; TISSUES; TRANSFERASES; VERTEBRATES
• ISSN: 0027-8424; 1091-6490
• DOI: 10.1073/pnas.88.12.5277

Cystic fibrosis (CF) involves a profound reduction of Cl{sup {minus}} permeability in several exocrine tissues. A distinctive, outwardly rectifying, depolarization-induced Cl{sup {minus}} channel (ORDIC channel) has been proposed to account for the Cl{sup {minus}} conductance that is defective in CF. The recently identified CF gene is predicted to code for a 1480-amino acid integral membrane protein termed the CF transmembrane conductance regulator (CFTR). The CFTR shares sequence similarity with a superfamily of ATP-binding membrane transport proteins such as P-glycoprotein and STE6, but it also has features consistent with an ion channel function. It has been proposed that the CFTR might be an ORDIC channel. To determine if CFTR and ORDIC channel expression are correlated, the authors surveyed various cell lines for natural variation in CFTR and ORDIC channel expression. In four human epithelial cell lines (T84, CaCo2, PANC-1, and 9HTEo-/S) that encompass the full observed range of CFTR mRNA levels and ORDIC channel density the authors found no correlation.