Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue

@inproceedings{Roy2010InterplayBE,
  title={Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue},
  author={Gargi Roy and Elaine M. Chalfin and Anita Saxena and Xiaodong Wang},
  booktitle={Molecular biology of the cell},
  year={2010}
}
Multiple mutations in cystic fibrosis transmembrane conductance regulator (CFTR) impair its exit from the endoplasmic reticulum (ER). We compared two processing mutants: DeltaF508 and the ER exit code mutant DAA. Although both have severe kinetic processing defect, DAA but not DeltaF508 has substantial accumulation in its mature form, leading to higher level of processing at the steady state. DAA has much less profound conformational abnormalities. It has lower Hsp70 association and higher post… CONTINUE READING

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