The role of the UPS in cystic fibrosis

  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},
  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

From This Paper

Topics from this paper.


Publications citing this paper.
Showing 1-10 of 32 extracted citations

Protein Ubiquitylation in Pancreatic Cancer

TheScientificWorldJournal • 2010
View 8 Excerpts
Highly Influenced

Targeting Trafficking in Drug Development

Handbook of Experimental Pharmacology • 2018


Publications referenced by this paper.
Showing 1-10 of 88 references

Cystic fibrosis.

The New England journal of medicine • 2005
View 6 Excerpts
Highly Influenced

Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis.

The Journal of clinical investigation • 2002
View 4 Excerpts
Highly Influenced

The ubiquitin-proteasome pathway: on protein death and cell life.

The EMBO journal • 1998
View 4 Excerpts
Highly Influenced

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.

American journal of physiology. Lung cellular and molecular physiology • 2006
View 9 Excerpts
Highly Influenced

Cystic fibrosis mouse models.

American journal of respiratory cell and molecular biology • 2007
View 1 Excerpt

Similar Papers

Loading similar papers…