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7 in total

1. Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo.

Authors: J Biwersi; N Emans; A S Verkman
Journal: Proc Natl Acad Sci U S A Date: 1996-10-29 Impact factor: 11.205

2. Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase.

Authors: K R Hallows; V Raghuram; B E Kemp; L A Witters; J K Foskett
Journal: J Clin Invest Date: 2000-06 Impact factor: 14.808

3. AMP-activated protein kinase phosphorylation of the R domain inhibits PKA stimulation of CFTR.

Authors: J Darwin King; Adam C Fitch; Jeffrey K Lee; Jill E McCane; Don-On Daniel Mak; J Kevin Foskett; Kenneth R Hallows
Journal: Am J Physiol Cell Physiol Date: 2009-05-06 Impact factor: 4.249

4. Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonist.

Authors: Wan Namkung; Jinhong Park; Yohan Seo; A S Verkman
Journal: Mol Pharmacol Date: 2013-06-20 Impact factor: 4.436

Cystic fibrosis: How do CFTR mutations cause cystic fibrosis?

1. Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo.

2. Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase.

3. AMP-activated protein kinase phosphorylation of the R domain inhibits PKA stimulation of CFTR.

4. Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonist.

5. Structural models of CFTR-AMPK and CFTR-PKA interactions: R-domain flexibility is a key factor in CFTR regulation.

6. Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines.

7. Inter-lab concordance of variant classifications establishes clinical validity of expanded carrier screening.