Protective Role of Endothelial Fibroblast Growth Factor Signaling in Group 3 Pulmonary Hypertension
Kel Vin Woo, M.D., Ph.D.
Center for Pediatric Pulmonary Disease, Congenital Heart Disease Center
2/1/2018 - 1/31/2020
Bronchopulmonary dysplasia (BPD) is a lung disease that can develop in pre-term infants, and in turn, lead to low-oxygen (hypoxic) conditions causing pulmonary hypertension (PH). Up to 25% of infants with BPD develop PH and face a two-year mortality rate of 33–48%. A protein called Fibroblast Growth Factor 2 [FGF2] and its receptors (FGFR 1 & 2) are elevated in BPD patients and PH patients. We will study how activation of these receptors can augment signals that protect against pulmonary hypertension, using mouse models and microfluidics devices
Proposed specific aims
· Determine how FGFR1/2 signaling affects the pathogenesis of hypoxia-induced PH.
· Investigate the effect of hypoxia and FGFR1/2 loss on endothelial cells lining the blood vessels and their adjacent smooth-muscle cells.
Potential impact on child health
This research will advance our understanding of how the FGF signaling pathway regulates PH and how that knowledge can be used to diagnose, prevent, or treat pediatric patients with BPD and PH.