Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.

@article{Lukacs1994ConformationalMO,
  title={Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.},
  author={Gergely L Lukacs and Ahmed Mohamed and Norbert Kartner and X Z Chang and John R. Riordan and Sergio Grinstein},
  journal={The EMBO journal},
  year={1994},
  volume={13 24},
  pages={6076-86}
}
Metabolic labeling experiments followed by immunoprecipitation were performed to investigate the kinetics, location and inhibitor sensitivity of degradation of both wild-type (wt) and mutant (delta F508) cystic fibrosis conductance transmembrane regulator (CFTR). At the earliest stages of the biosynthetic process, both wt and delta F508 CFTR were found to be susceptible to degradation by endogenous proteases. Virtually all delta F508 CFTR and 45-80% of wt CFTR were rapidly degraded with a… CONTINUE READING

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