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A Stabilizing Influence: CAL PDZ Inhibition Extends the Half‐Life of ΔF508‐CFTR

Year: 2010
Type: article
Abstract: It pays to recycle! The most common mutation in cystic fibrosis (CF) impedes maturation and accelerates breakdown of the ion channel CFTR. A “stabilizer” has been characterized that blocks a PDZ domain responsible for CFTR degradation. The inhibitor iCAL36 extends the chloride channel's half-life in airway epithelial cells. It also complements the activity of a corrector of the maturation defect, thus suggesting the potential for combination CF therapies.
Language: English
Field: Medicine
Open Access status: green
OpenAlex ID: W2123987449
FWCI: 6.135
Cites: 35
Cited by: 66
Related to: 10