Related Experiment Video
Updated: Aug 21, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
Altered terminal glycosylation and the pathophysiology of CF lung disease
Andrew D Rhim1, Lidia I Stoykova, Arvind J Trindade
1Cystic Fibrosis Center and Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, ARB 402, 34th and Civic Center Blvd., Philadelphia, PA 19104, USA.
Abstract:
Altered terminal glycosylation, with increased fucosylation and decreased sialylation, is a hallmark of the cystic fibrosis (CF) glycosylation phenotype. The glycosylation phenotype of CF airway epithelial cells has been modulated by the expression of wtCFTR. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as new approaches to the therapy of CF.
Related Concept Videos
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cirrhosis II: Pathophysiology
Chronic Pancreatitis II: Pathophysiology
