The top 10 research priorities in cystic fibrosis developed by a partnership between people with CF and healthcare providers [PDF]
There remain many treatment uncertainties in cystic fibrosis (CF). With limited resources, research should focus on questions which are most important to the CF community. We conducted a James Lind Alliance Priority Setting Partnership in CF.
Alan R Smyth +20 more
core +2 more sources
The potential for biased signalling in the P2Y receptor family of GPCRs
The purinergic receptor family is primarily activated by nucleotides, and contains members of both the G protein coupled‐receptor (GPCR) superfamily (P1 and P2Y) and ligand‐gated ion channels (P2X). The P2Y receptors are widely expressed in the human body, and given the ubiquitous nature of nucleotides, purinergic signalling is involved with a plethora
Claudia M. Sisk +2 more
wiley +1 more source
Two Small Molecules Restore Stability to a Sub-population of the Cystic Fibrosis Transmembrane conductance Regulator with the Predominant Disease-causing Mutation [PDF]
Cystic fibrosis (CF) is caused by mutations that disrupt the plasma membraneexpression, stability, and function of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Cai, Zhiwei +8 more
core +1 more source
Abstract Background Phenylbutazone (PBZ) can potentially induce gastrointestinal ulceration, and early detection of PBZ‐induced gastroenteropathy will be useful for the diagnosis, treatment, and prevention of PBZ toxicity. Objectives To identify putative proteins associated with equine gastric ulcer syndrome after clinical dose (4.4 mg/kg ...
Ruethaiwan Vinijkumthorn +6 more
wiley +1 more source
The function and regulation of acid-sensing ion channels (ASICs) and the epithelial Na(+) channel (ENaC): IUPHAR Review 19. [PDF]
Acid-sensing ion channels (ASICs) and the epithelial Na(+) channel (ENaC) are both members of the ENaC/degenerin family of amiloride-sensitive Na(+) channels.
Alijevic, O. +4 more
core +1 more source
TDP‐43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models
The manuscript explores structural and functional features of TDP‐43 and its worm homologue, TDP‐1, highlighting conserved and divergent structural and functional features. Using genetically engineered C. elegans models, key pathological features of TDP‐43 proteinopathies—including aggregation, neurodegeneration, and motor deficits—are recapitulated ...
Ghulam Jeelani Pir +7 more
wiley +1 more source
Lung organoids as a human system for Mycobacteria infection modeling and drug testing
Mycobacterial infections, including tuberculosis (TB) and infections by nontuberculous mycobacteria (NTM), are still public health issues. In 2023, TB caused 1.25 million deaths, while NTM remain a clinical challenge for patients with cystic fibrosis (CF).
Stephen Adonai Leon‐Icaza +4 more
wiley +1 more source
Over the last years CFTR (cystic fibrosis transmembrane conductance regulator) modulators have shown the ability to improve relevant clinical outcomes in patients with cystic fibrosis (CF).
A. Gramegna +5 more
semanticscholar +1 more source
How Should the Effects of CFTR Modulator Therapy on Cystic Fibrosis Lung Disease Be Monitored?
Elad Ben Meir, Hartmut Grasemann
openalex +1 more source
Abstract Pancreatic ductal adenocarcinoma (PDAC) is an aggressive malignancy with a dismal prognosis, largely because of late‐stage diagnosis and therapeutic resistance. PDAC incidence has been rising, with modifiable and non‐modifiable risk factors contributing to disease development.
Alessandro Mannucci, Ajay Goel
wiley +1 more source

