The role of the UPS in cystic fibrosis
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-rel...
| Main Authors: | , , |
|---|---|
| Format: | Online |
| Language: | English |
| Published: |
BioMed Central
2007
|
| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2106362/ |