Results 41 to 50 of about 156,262 (248)
The Journal of the Royal College of Physicians of Edinburgh, 2013 There are now more adult than paediatric cystic fibrosis (CF) patients and their life expectancy continues to improve. This means that CF patients will be more commonly encountered in a variety of hospital settings including fertility services, gastrointestinal (GI) clinics, diabetes clinics, surgical wards, and acute admissions.
A E, Leitch, H C, Rodgers
openaire +2 more sources
Advanced Science, EarlyView.Expanded NCAM1+EpCAM+ hepatic progenitor cells in biliary atresia are characterized by aggregation of α‐synuclein. This pathological protein potentiates cellular susceptibility to GSH‐dependent redox dyshomeostasis, induces unstable biliary cell fate specification, and subsequently drives aberrant biliary regeneration.
Hua Xie +12 more
wiley +1 more source
A multimodal iPSC platform for cystic fibrosis drug testing
Nature Communications, 2022 Hundreds of mutations in the gene CFTR lead to cystic fibrosis and represent a challenge to developing therapeutics. Here, authors demonstrate the ability of airway cells derived from human iPSCs to model genotype-specific CFTR function as well as ...
Andrew Berical +20 more
doaj +1 more source
Advanced Science, EarlyView.An integrated Human Airway Microbiome Gene Catalog (iHAMGC) is constructed from 12,273 metagenomic airway samples, providing a high‐resolution reference of respiratory microbial genes. The catalog enables systematic exploration of taxonomic and functional variation across airway niches and geographic regions, revealing site‐specific and region ...
Qing Zhang +15 more
wiley +1 more source
Cystic fibrosis and the role of the physiotherapist
South African Journal of Physiotherapy, 1981 Cystic fibrosis is an inherited disease, the major problems being pancreatic malabsorption and progressive respiratory involvement, although numerous other organs may also be involved.
Anne Miot, J. M. Pettifor, I. Reef
doaj +1 more source
Long term respiratory morbidity in patients with vascular rings: a review
Italian Journal of Pediatrics, 2023 Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the ...
Federica Porcaro +6 more
doaj +1 more source
Therapeutic Gene Editing of APOE4 in Sporadic Alzheimer's Disease via Prime Editor 7
Advanced Science, EarlyView.Prime Editor 7‐mediated conversion of APOE4 to APOE3 alleviates Alzheimer's disease‐associated pathology in AD mouse models and patient‐derived neurons and improves cognitive performance in vivo, supporting therapeutic genome editing as a promising strategy for APOE4‐associated neurodegeneration.
Yunkyung Kim +16 more
wiley +1 more source
Advanced Science, EarlyView.This study identifies that the PD‐associated TMEM175‐L156P variant disrupts lysosomal ion channel trafficking by causing aberrant endoplasmic reticulum retention. A “chaperone–agonist” bifunctional small molecule restores TMEM175‐L156P lysosomal localization and channel function, thereby alleviating PD‐relevant cellular phenotypes and highlighting a ...
Ting Luo +17 more
wiley +1 more source
Frontiers in NutritionBackgroundWhen people with cystic fibrosis (PwCFs) are treated with cystic fibrosis transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition.
Ana Merino Sánchez-Cañete +6 more
doaj +1 more source