cystic fibrosis

[Nature Communications] Scientists generated functional human PSC-derived cholangiocytes that displayed many characteristics of mature bile duct cells including high levels of cystic fibrosis transmembrane conductance regulator and the presence of primary cilia capable of sensing flow.

[ISME Journal] The bacterial communities in 818 sputum samples from 109 persons with cystic fibrosis were analyzed by sequencing bacterial 16S rRNA gene amplicons. Researchers identified eight alternative community types or pulmotypes by using a Dirichlet multinomial mixture model and studied their temporal dynamics in the cohort.

[Journal of Cystic Fibrosis] The authors assessed induction of cystic fibrosis transmembrane conductance regulator (CFTR) function in premature termination codons-containing intestinal organoids using compounds targeting readthrough, nonsense mRNA mediated decay and CFTR protein modulation.

[American Journal of Physiology-Cell Physiology] Researchers investigated how culture of normal human bronchial epithelial cells with apically applied secretions from healthy or disease lungs affected epithelial function with a view to providing better in vitro models of the in vivo environment.

[American Journal of Physiology-Cell Physiology] Scientists generated 3D esophageal organoids (EOs) from two different mouse strains and characterized the ion transport processes of the EOs.

[Scientific Reports] Scientists investigated the role of Splicing factor proline/glutamine-rich (SFPQ) in the regulation of the expression and function of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) in cystic fibrosis lung epithelial cells.