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Showing 1–6 of 6 results
Advanced filters: Author: Lynda Ostedgaard Clear advanced filters

  • The cystic fibrosis mutation, ΔF508, disrupts CFTR biosynthesis. ΔF508 protein resembles an early intermediate in wild type folding, but fails to adopt a mature conformation and hence cannot escape the ER.

    • Michael J. Welsh
    • Lynda S. Ostedgaard
    News & Views
    Nature Structural Biology
    Volume: 5, P: 167-169

  • The ATP-binding cassette (ABC) transporter family of proteins all contain two nucleotide-binding domains (NBDs) along with two membrane-spanning domains. The NBDs are molecular engines, and the crystal structure of one -- the NBD from theSalmonella typhimuriumhistidine permease -- now gives us clues about how the NBDs work. The results have implications for other ABC transporters, including the protein that is mutated in cystic fibrosis.

    • Michael J. Welsh
    • Andrew D. Robertson
    • Lynda S. Ostedgaard
    News & Views
    Nature
    Volume: 396, P: 623-624

  • In this study, the authors assess the early pathogenesis of cystic fibrosis (CF) pig gallbladder disease. The CF pig gallbladder epithelium lacks cAMP-stimulated anion and fluid transport. CF pig gallbladders also demonstrate increased luminal mucins MUC5AC and MUC5B accumulation without significant changes in the epithelial expression of gel-forming mucins compared to non-CF pigs.

    • Keyan Zarei
    • Mallory R. Stroik
    • David A. Stoltz
    Research
    Laboratory Investigation
    Volume: 100, P: 1388-1399