Increased pulmonary vascular expression of receptor for advanced glycation end products (RAGE) in experimental congenital diaphragmatic hernia

Journal of Pediatric Surgery
Alejandro HofmannPrem Puri

Abstract

Persistent pulmonary hypertension (PH) continues to be a major cause of high mortality in congenital diaphragmatic hernia (CDH). The receptor for advanced glycation end products (RAGE) is a member of the immunoglobulin protein family. Recently, RAGE has been implicated in mediating pulmonary arterial smooth muscle cell proliferation and vascular remodeling in experimental PH. RAGE has been reported to be highly upregulated in lung tissue of patients with severe PH. We designed this study to investigate the hypothesis that RAGE expression is increased in nitrofen-induced CDH. Pregnant rats were exposed to nitrofen or vehicle on D9. Fetuses were sacrificed on D21 and divided into nitrofen (n=16) and control group (n=16). Quantitative real-time polymerase chain reaction, Western blotting, and confocal immunofluorescence were performed. Pulmonary RAGE gene expression levels were significantly increased in nitrofen-induced CDH compared to controls (p<0.003). Western blotting and confocal microscopy revealed increased pulmonary RAGE protein expression in CDH compared to controls. This study provides striking evidence of increased gene and protein expression of RAGE in the pulmonary vasculature of nitrofen-induced CDH, suggesting that...Continue Reading

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Citations

Mar 7, 2019·American Journal of Physiology. Lung Cellular and Molecular Physiology·Florian KipfmuellerStefan Holdenrieder
Nov 2, 2020·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Daniel PrantnerStefanie N Vogel

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