Arun Jose, Jean M. Elwing, Steven M. Kawut, Michael W. Pauciulo, Kenneth E. Sherman, William C. Nichols, Michael B. Fallon, Francis X. McCormack
University of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center. Perelman School at the University of Pennsylvania. University of Arizona.
United States
Communications Biology
Commun Biol 2023; 6
DOI: 10.1038/s42003-023-05193-3
Abstract
Portopulmonary hypertension (PoPH) is a type of pulmonary vascular disease due to portal hypertension that exhibits high morbidity and mortality. The mechanisms driving disease are unknown, and transcriptional characteristics unique to the PoPH liver remain unexplored. Here, we apply single nuclear RNA sequencing to compare cirrhotic livers from patients with and without PoPH. We identify characteristics unique to PoPH in cells surrounding the central hepatic vein, including increased growth differentiation factor signaling, enrichment of the arginine biosynthesis pathway, and differential expression of the bone morphogenic protein type II receptor and estrogen receptor type I genes. These results provide insight into the transcriptomic characteristics of the PoPH liver and mechanisms by which PoPH cellular dysfunction might contribute to pulmonary vascular remodeling.
Category
Class I. Pulmonary Hypertension Associated with Liver Disease
Vascular Cell Biology and Mechanisms of Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access Free PDF File or Full Text Article Available Through PubMed or DOI: Yes