Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.

@article{Aleksandrov2010RegulatoryIR,
  title={Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.},
  author={Andrei A. Aleksandrov and Pradeep Kota and Luba A. Aleksandrov and Lihua He and Tim Joachim Jensen and Li-ying Cui and Martina Gentzsch and Nikolay V. Dokholyan and John R. Riordan},
  journal={Journal of molecular biology},
  year={2010},
  volume={401 2},
  pages={194-210}
}
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel is a large multidomain membrane protein that matures inefficiently during biosynthesis. Its assembly is further perturbed by the deletion of F508 from the first nucleotide-binding domain (NBD1) responsible for most cystic fibrosis. The mutant polypeptide is recognized by cellular quality control systems and is proteolyzed. CFTR NBD1 contains a 32-residue segment termed the regulatory insertion (RI) not… CONTINUE READING
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