Targeting of carbonic anhydrase IV to plasma membranes is altered in cultured human pancreatic duct cells expressing a mutated (ΔF508) CFTR

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

Seng H. Cheng, Richard J. Gregory, John Marshall et al. · Cell · 1990 · 1.8K citations

Fibrosis, Molecular Physiology, Defective Intracellular Transport +8

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

Timothy J. Jensen, Melinda A. Loo, S. Pind et al. · Cell · 1995 · 892 citations · Full text

Protease Inhibitors, Protein Function, Cftr Processing +10

Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

Mitchell L. Drumm, Heidi A. Pope, William Cliff et al. · Cell · 1990 · 575 citations · Full text

In Vivo Gene Therapy, Retrovirus-mediated Gene Transfer, Reverse Genetics +11

Defective acidification of intracellular organelles in cystic fibrosis

Jonathan Barasch, Brian Kiss, Alice Prince et al. · Nature · 1991 · 487 citations

Organelle Dynamic, Medicine, Cell Biology +2

Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.

Joo Hanne Poulsen, Horst Fischer, Beate Illek et al. · Proceedings of the National Academy of Sciences · 1994 · 416 citations · Full text

Cellular Physiology, Membrane Transport, Phi Recovery +19