Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function.

@article{Howard2003MammalianOA,
  title={Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function.},
  author={Marybeth Howard and Horst Fischer and J{\'e}r{\'e}mie Roux and Bento C. Santos and Steven R. Gullans and Paul H. Yancey and William J. Welch},
  journal={The Journal of biological chemistry},
  year={2003},
  volume={278 37},
  pages={
          35159-67
        }
}
In cystic fibrosis, the absence of functional CFTR results in thick mucous secretions in the lung and intestines, as well as pancreatic deficiency. Although expressed at high levels in the kidney, mutations in CFTR result in little or no apparent kidney dysfunction. In an effort to understand this phenomenon, we analyzed Delta F508 CFTR maturation and function in kidney cells under conditions that are common to the kidney, namely osmotic stress. Kidney cells were grown in culture and adapted to… CONTINUE READING

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