Disease-associated Mutations in the Extracytoplasmic Loops of Cystic Fibrosis Transmembrane Conductance Regulator Do Not Impede Biosynthetic Processing but Impair Chloride Channel Stability
Open Access
- 1 January 2001
- journal article
- Published by Elsevier
- Vol. 276 (18), 14848-14854
- https://doi.org/10.1074/jbc.m011017200
Abstract
No abstract availableKeywords
This publication has 17 references indexed in Scilit:
- Biosynthesis and Degradation of CFTRPhysiological Reviews, 1999
- Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasomeThe EMBO Journal, 1998
- Regulation of CFTR ion channel gating by MgATPFEBS Letters, 1998
- Disease-Associated Mutations in Cytoplasmic Loops 1 and 2 of Cystic Fibrosis Transmembrane Conductance Regulator Impede Processing or Opening of the ChannelBiochemistry, 1997
- Cytoplasmic Loop Three of Cystic Fibrosis Transmembrane Conductance Regulator Contributes to Regulation of Chloride Channel ActivityJournal of Biological Chemistry, 1996
- Disease-associated Mutations in the Fourth Cytoplasmic Loop of Cystic Fibrosis Transmembrane Conductance Regulator Compromise Biosynthetic Processing and Chloride Channel ActivityPublished by Elsevier ,1996
- Conformational states of CFTR associated with channel gating: The role of ATP binding and hydrolysisCell, 1995
- cAMP-dependent Protein Kinase-mediated Phosphorylation of Cystic Fibrosis Transmembrane Conductance Regulator Residue Ser-753 and Its Role in Channel ActivationPublished by Elsevier ,1995
- Mutations in CFTR associated with mild-disease-form CI- channels with altered pore propertiesNature, 1993
- Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNAScience, 1989