The role of the UPS in cystic fibrosis

@article{Turnbull2007TheRO,
  title={The role of the UPS in cystic fibrosis},
  author={Emma L. Turnbull and Meredith F N Rosser and Douglas M. Cyr},
  journal={BMC Biochemistry},
  year={2007},
  volume={8},
  pages={S11 - S11}
}
CF is an inherited autosomal recessive disease whose lethality arises from malfunction of CFTR, a single chloride (Cl-) ion channel protein. CF patients harbor mutations in the CFTR gene that lead to misfolding of the resulting CFTR protein, rendering it inactive and mislocalized. Hundreds of CF-related mutations have been identified, many of which abrogate CFTR folding in the endoplasmic reticulum (ER). More than 70% of patients harbor the ΔF508 CFTR mutation that causes misfolding of the CFTR… CONTINUE READING

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