The ΔF508 Mutation Disrupts Packing of the Transmembrane Segments of the Cystic Fibrosis Transmembrane Conductance Regulator

This publication has 56 references indexed in Scilit:

• The Phenotypic Consequences of CFTR Mutations
  Annals of Human Genetics, 2003
• Therapeutic approaches to repair defects in ΔF508 CFTR folding and cellular targeting
  Advanced Drug Delivery Reviews, 2002
• The PEST sequence does not contribute to the stability of the cystic fibrosis transmembrane conductance regulator
  BMC Biochemistry, 2002
• Cystic Fibrosis Transmembrane Conductance Regulator Has an Altered Structure When Its Maturation Is Inhibited¹
  Biochemistry, 2000
• Disease-Associated Mutations in Cytoplasmic Loops 1 and 2 of Cystic Fibrosis Transmembrane Conductance Regulator Impede Processing or Opening of the Channel
  Biochemistry, 1997
• Disease-associated Mutations in the Fourth Cytoplasmic Loop of Cystic Fibrosis Transmembrane Conductance Regulator Compromise Biosynthetic Processing and Chloride Channel Activity
  Published by Elsevier ,1996
• Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
  Cell, 1995
• Degradation of CFTR by the ubiquitin-proteasome pathway
  Cell, 1995
• Epithelial Cell Dysfunction in Cystic Fibrosis: Implications for Airways Disease
  Acta Paediatrica, 1989
• Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
  Science, 1989