Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.

  title={Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.},
  author={Andrei A. Aleksandrov and Pradeep Kota and Li-ying Cui and Tim Joachim Jensen and Alexey Alekseev and Santiago Reyes and Lihua He and Martina Gentzsch and Luba A. Aleksandrov and Nikolay V. Dokholyan and John R. Riordan},
  journal={Journal of molecular biology},
  volume={419 1-2},
Most cystic fibrosis is caused by a deletion of a single residue (F508) in CFTR (cystic fibrosis transmembrane conductance regulator) that disrupts the folding and biosynthetic maturation of the ion channel protein. Progress towards understanding the underlying mechanisms and overcoming the defect remains incomplete. Here, we show that the thermal instability of human ΔF508 CFTR channel activity evident in both cell-attached membrane patches and planar phospholipid bilayers is not observed in… CONTINUE READING


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