The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a disease that causes scarring and fibrotic transformation of the lung parenchyma, resulting in the progressive loss of respiratory function and, often, death. Current treatments that target profibrotic factors can slow the rate of progression but are unable to...
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MDPI AG
2023-06-01
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Series: | Biomedicines |
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Online Access: | https://www.mdpi.com/2227-9059/11/6/1728 |
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author | Girish Jayant Stephen Kuperberg Kaumudi Somnay Raj Wadgaonkar |
author_facet | Girish Jayant Stephen Kuperberg Kaumudi Somnay Raj Wadgaonkar |
author_sort | Girish Jayant |
collection | DOAJ |
description | Idiopathic pulmonary fibrosis (IPF) is a disease that causes scarring and fibrotic transformation of the lung parenchyma, resulting in the progressive loss of respiratory function and, often, death. Current treatments that target profibrotic factors can slow the rate of progression but are unable to ultimately stop it. In the past decade, many studies have shown that increased vascular permeability may be both a predictive and perpetuating factor in fibrogenesis. Consequently, there is a search for therapeutic targets to try and modulate vascular permeability in fibrotic lungs. One such class of targets that show great promise is sphingolipids. Sphingolipids are common in cell membranes and are increasingly recognized as critical to many cell signaling pathways, including those that affect the integrity of the vascular endothelial barrier. In this focused review we look at sphingolipids, particularly the sphingosine-1-phosphate (S1P) axis and its effects on vascular permeability, and how those effects may affect the pathogenesis of IPF. We further examine existing S1P modulators and their potential efficacy as therapeutics for IPF. |
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issn | 2227-9059 |
language | English |
last_indexed | 2024-03-11T02:44:17Z |
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spelling | doaj.art-f44c571c0d3341229744fab5223e8d7b2023-11-18T09:27:21ZengMDPI AGBiomedicines2227-90592023-06-01116172810.3390/biomedicines11061728The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary FibrosisGirish Jayant0Stephen Kuperberg1Kaumudi Somnay2Raj Wadgaonkar3SUNY Downstate College of Medicine, Brooklyn, NY 11203, USANYU Grossman School of Medicine, New York, NY 10016, USANY Presbyterian Hospital Queens, New York, NY 11355, USASUNY Downstate College of Medicine, Brooklyn, NY 11203, USAIdiopathic pulmonary fibrosis (IPF) is a disease that causes scarring and fibrotic transformation of the lung parenchyma, resulting in the progressive loss of respiratory function and, often, death. Current treatments that target profibrotic factors can slow the rate of progression but are unable to ultimately stop it. In the past decade, many studies have shown that increased vascular permeability may be both a predictive and perpetuating factor in fibrogenesis. Consequently, there is a search for therapeutic targets to try and modulate vascular permeability in fibrotic lungs. One such class of targets that show great promise is sphingolipids. Sphingolipids are common in cell membranes and are increasingly recognized as critical to many cell signaling pathways, including those that affect the integrity of the vascular endothelial barrier. In this focused review we look at sphingolipids, particularly the sphingosine-1-phosphate (S1P) axis and its effects on vascular permeability, and how those effects may affect the pathogenesis of IPF. We further examine existing S1P modulators and their potential efficacy as therapeutics for IPF.https://www.mdpi.com/2227-9059/11/6/1728idiopathic pulmonary fibrosissphingolipidssphingosine-1-phosphatevascular permeabilityendothelial barrier dysfunction |
spellingShingle | Girish Jayant Stephen Kuperberg Kaumudi Somnay Raj Wadgaonkar The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis Biomedicines idiopathic pulmonary fibrosis sphingolipids sphingosine-1-phosphate vascular permeability endothelial barrier dysfunction |
title | The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis |
title_full | The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis |
title_fullStr | The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis |
title_full_unstemmed | The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis |
title_short | The Role of Sphingolipids in Regulating Vascular Permeability in Idiopathic Pulmonary Fibrosis |
title_sort | role of sphingolipids in regulating vascular permeability in idiopathic pulmonary fibrosis |
topic | idiopathic pulmonary fibrosis sphingolipids sphingosine-1-phosphate vascular permeability endothelial barrier dysfunction |
url | https://www.mdpi.com/2227-9059/11/6/1728 |
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