Cystic fibrosis (CF) is an autosomal recessive disease that was previously a fatal pediatric disease with no treatment; however, due to scientific advancements, the median age of survival for the CF population born in 2018 has increased from 29 in 1989 to 47.4 in 2018. This is an innovative era for the treatment of CF as advanced research continues to evolve and novel treatments for the disease and related illnesses are discovered.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7721430 | PMC |
Publication Analysis
Top Keywords
Similar Publications
Inhibiting CFTR through inh-172 in primary neutrophils reveals CFTR-specific functional defects.
Sci Rep
December 2024
Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism, KU Leuven, Leuven, Belgium.
The lungs of people with cystic fibrosis (PwCF) are characterized by recurrent bacterial infections and inflammation. Infections in cystic fibrosis (CF) are left unresolved despite excessive neutrophil infiltration. The role of CFTR in neutrophils is not fully understood.
View Article and Find Full Text PDFPossible drug-interaction between elexacaftor-tezacaftor-ivacaftor and repaglinide in an adult with cystic fibrosis-related diabetes.
Can J Diabetes
December 2024
Division of Endocrinology & Metabolism, Department of Medicine, Nova Scotia Health. QEII - Victoria Building, Suite 7-North-046 Victoria Building, 1276 South Park Street, Halifax, Nova Scotia, Canada, B3H 2Y9.
Disorder within order: identification of the disordered loop of STAS domain as the inhibitory domain in SLC26A9 chloride channel.
J Biol Chem
December 2024
Physiology & Biomedical Engineering, Mayo Clinic College of Medicine & Science, Rochester, MN, 55906; Nephrology & Hypertension, Mayo Clinic College of Medicine & Science, Rochester, MN, 55906. Electronic address:
The chloride transporter-channel SLC26A9 is mediated by a reciprocal regulatory mechanism through the interaction between its cytoplasmic STAS domain and the R domain of CFTR. In vertebrate Slc26a9s, the STAS domain structures are interrupted by a disordered loop which is conserved in mammals but is variable in non-mammals. Despite the numerous studies involving the STAS domains in SLC26 proteins, the role of the disordered loop region has not been identified.
View Article and Find Full Text PDFMesenchymal stromal cells reduce inflammation and improve lung function in a mouse model of cystic fibrosis lung disease.
Sci Rep
December 2024
Airway Innate Immunity Research Group, Wellcome-Wolfson Institute for Experimental Medicine, Queen's University, Belfast, UK.
Mesenchymal stromal cells (MSCs) are multipotent adult stem cells which possess immunomodulatory and repair capabilities. In this study, we investigated whether MSC therapy could modulate inflammation and lung damage in the lungs of Scnn1b-transgenic mice overexpressing the β-subunit of the epithelial sodium channel (β-ENaC), a model with features of Cystic Fibrosis lung disease. Human bone marrow derived MSC cells were intravenously delivered to mice, prior to collection of bronchoalveolar lavage (BALF) and tissue.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!