Do you know the sex of your cells?
K Shah, CE McCormack… - American Journal of …, 2014 - journals.physiology.org
Do you know the sex of your cells? Not a question that is frequently heard around the lab
bench, yet thanks to recent research is probably one that should be asked. It is self-evident …
bench, yet thanks to recent research is probably one that should be asked. It is self-evident …
Chaperoning G protein-coupled receptors: from cell biology to therapeutics
YX Tao, PM Conn - Endocrine reviews, 2014 - academic.oup.com
G protein-coupled receptors (GPCRs) are membrane proteins that traverse the plasma
membrane seven times (hence, are also called 7TM receptors). The polytopic structure of …
membrane seven times (hence, are also called 7TM receptors). The polytopic structure of …
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 …
Background The phe508del CFTR mutation causes cystic fibrosis by limiting the amount of
CFTR protein that reaches the epithelial cell surface. We tested combination treatment with …
CFTR protein that reaches the epithelial cell surface. We tested combination treatment with …
[HTML][HTML] Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function
F Van Goor, H Yu, B Burton, BJ Hoffman - Journal of Cystic Fibrosis, 2014 - Elsevier
Abstract Background Ivacaftor (KALYDECO™, VX-770) is a CFTR potentiator that increased
CFTR channel activity and improved lung function in patients age 6 years and older with CF …
CFTR channel activity and improved lung function in patients age 6 years and older with CF …
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression
G Veit, RG Avramescu, D Perdomo… - Science translational …, 2014 - science.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane regulator (CFTR) that
result in reduced anion conductance at the apical membrane of secretory epithelia …
result in reduced anion conductance at the apical membrane of secretory epithelia …
CFTR: cystic fibrosis and beyond
MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
DM Cholon, NL Quinney, ML Fulcher… - Science translational …, 2014 - science.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR …
(CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR …
[HTML][HTML] Glucose-induced electrical activities and insulin secretion in pancreatic islet β-cells are modulated by CFTR
JH Guo, H Chen, YC Ruan, XL Zhang… - Nature …, 2014 - nature.com
The cause of insulin insufficiency remains unknown in many diabetic cases. Up to 50% adult
patients with cystic fibrosis (CF), a disease caused by mutations in the gene encoding the …
patients with cystic fibrosis (CF), a disease caused by mutations in the gene encoding the …
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
DD Stefano, VR Villella, S Esposito, A Tosco, A Sepe… - Autophagy, 2014 - Taylor & Francis
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by
genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine …
genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine …
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor
New drugs are needed to enhance premature termination codon (PTC) suppression to treat
the underlying cause of cystic fibrosis (CF) and other diseases caused by nonsense …
the underlying cause of cystic fibrosis (CF) and other diseases caused by nonsense …