Yohei Akazawa, Sriram Vaidyanathan, Michael Bradley Dewar, Kana Yazaki, John Dauz, Osami Honjo, Mei Sun, Golam Kabir, Robert P. Jankov, Scott Patrick Heximer, Kim A. Connelly, Mark K. Friedberg
Hospital for Sick Children and University of Toronto. Stanford University. St. Michael’s Hospital. Children’s Hospital of Eastern Ontario Research Institute.
Canada and United States
Journal of the American College of Cardiology Basic to Translational Science
JACC Basic Transl Sci 2025; 10: 323-341
DOI: 10.1016/j.jacbts.2024.10.007
Abstract
Right ventricular (RV) pressure loading induces RV profibrotic signaling and fibrosis associated with RV dysfunction. RV decorin protein levels are decreased in patients with chronic RV pressure loading. RV decorin protein levels are also decreased in 4 animal models of mechanical RV pressure loading and pulmonary arterial hypertension. Human cardiac fibroblasts overexpressing decorin show diminished collagen-1 secretion in response to mechanical or chemical profibrotic stress while decorin knockout human cardiac fibroblasts show increased collagen-1 secretion in response to stress. Downregulation of decorin may play a key role in upregulating transforming growth factor-β1 profibrotic signaling and fibrosis that contribute to RV dysfunction in RV pressure loading.
Category
Right Heart Dysfunction Associated with Pulmonary Vascular Disease
Animal Models of Pulmonary Vascular Disease and Therapy
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes