Article
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
Nature - 27 Aug 1992
Denning G M, Anderson M P, Amara J F, Marshall J, Smith A E, Welsh M J
Abstract excerpt
Cystic fibrosis transmembrane conductance regulator (CFTR) is a plasma membrane Cl- channel regulated by cyclic AMP-dependent phosphorylation and by intracellular ATP. Mutations in CFTR cause cystic fibrosis partly through loss of cAMP-regulated Cl- permeability from the plasma membrane of affected epithelia. The most common mutation in cystic fibrosis is deletion of phenylalanine at residue 508 (CFTR delta F508)...
Topics
- 3T3 Cells
- Animals
- Cell Compartmentation
- Cell Membrane
- Chloride Channels
- Cold Temperature
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Glycosylation
- Humans
- In Vitro Techniques