cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with no effective treatment. Myofibroblasts contribute to the pathology of IPF by secreting large amounts of extracellular matrix proteins such as alpha smooth muscle actin (α-SMA) and Collagen I (Col 1). Myofibroblasts have reduced Pros...

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Main Author: Wright, Rebecca
Format: Thesis (University of Nottingham only)
Language:English
Published: 2016
Subjects:
Online Access:https://eprints.nottingham.ac.uk/35925/
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author Wright, Rebecca
author_facet Wright, Rebecca
author_sort Wright, Rebecca
building Nottingham Research Data Repository
collection Online Access
description Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with no effective treatment. Myofibroblasts contribute to the pathology of IPF by secreting large amounts of extracellular matrix proteins such as alpha smooth muscle actin (α-SMA) and Collagen I (Col 1). Myofibroblasts have reduced Prostaglandin E2 (PGE2), a key anti-fibrotic mediator, due to diminished cyclooxygenase-2 (COX-2) expression. Primary fibroblasts isolated from lungs of IPF patients (F-IPF) expressed significantly less COX-2 in response to IL-1β and increased α-SMA and Col I compared with fibroblasts isolated from lungs of non-fibrotic patients (F-NL). COX-2 was gradually lost in F-NL treated with transforming growth factor-β (TGF-β1), a pro-fibrotic cytokine, whereas PGE2, and cAMP elevating agents increased IL-1β-induced COX-2 expression in F-IPF. Ras, a small G protein, has been shown to have a role in several fibrotic conditions. Farnesylthiosalicylic acid (FTS), a Ras inhibitor, increased IL-1β-induced COX-2 and prevented TGF-β1-induced reduction of COX-2. Previous studies suggest that COX-2 is epigenetically repressed. LBH589, a HDAC inhibitor, prevented TGF-β1-induced repressed COX-2 whereas BIX01294, a DNA lysine methyltransferase inhibitor, and RG108, a G9a histone methyltransferase inhibitor, both increased IL-1β-induced COX-2 in F-IPF. In conclusion, the gradual loss of PGE2/COX-2 anti-fibrotic mechanism during myofibroblast differentiation may contribute to the pathophysiology of pulmonary fibrosis and agents that increase cAMP levels, inhibit Ras or inhibit epigenetic repression of COX-2, may compensate for the lack of endogenous PGE2.
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spelling nottingham-359252025-02-28T11:50:33Z https://eprints.nottingham.ac.uk/35925/ cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis Wright, Rebecca Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with no effective treatment. Myofibroblasts contribute to the pathology of IPF by secreting large amounts of extracellular matrix proteins such as alpha smooth muscle actin (α-SMA) and Collagen I (Col 1). Myofibroblasts have reduced Prostaglandin E2 (PGE2), a key anti-fibrotic mediator, due to diminished cyclooxygenase-2 (COX-2) expression. Primary fibroblasts isolated from lungs of IPF patients (F-IPF) expressed significantly less COX-2 in response to IL-1β and increased α-SMA and Col I compared with fibroblasts isolated from lungs of non-fibrotic patients (F-NL). COX-2 was gradually lost in F-NL treated with transforming growth factor-β (TGF-β1), a pro-fibrotic cytokine, whereas PGE2, and cAMP elevating agents increased IL-1β-induced COX-2 expression in F-IPF. Ras, a small G protein, has been shown to have a role in several fibrotic conditions. Farnesylthiosalicylic acid (FTS), a Ras inhibitor, increased IL-1β-induced COX-2 and prevented TGF-β1-induced reduction of COX-2. Previous studies suggest that COX-2 is epigenetically repressed. LBH589, a HDAC inhibitor, prevented TGF-β1-induced repressed COX-2 whereas BIX01294, a DNA lysine methyltransferase inhibitor, and RG108, a G9a histone methyltransferase inhibitor, both increased IL-1β-induced COX-2 in F-IPF. In conclusion, the gradual loss of PGE2/COX-2 anti-fibrotic mechanism during myofibroblast differentiation may contribute to the pathophysiology of pulmonary fibrosis and agents that increase cAMP levels, inhibit Ras or inhibit epigenetic repression of COX-2, may compensate for the lack of endogenous PGE2. 2016-12-16 Thesis (University of Nottingham only) NonPeerReviewed application/pdf en arr https://eprints.nottingham.ac.uk/35925/1/Thesis%20-%20FINAL%20CORRECTIONS%2020_05_2016%20%28changes%20highlighted%29.pdf Wright, Rebecca (2016) cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis. PhD thesis, University of Nottingham. Pulmonary fibrosis Myofibroblasts PGE2 cAMP levels
spellingShingle Pulmonary fibrosis
Myofibroblasts
PGE2
cAMP levels
Wright, Rebecca
cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title_full cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title_fullStr cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title_full_unstemmed cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title_short cAMP mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
title_sort camp mediated regulation of fibroblast to myofibroblast differentiation in idiopathic pulmonary fibrosis
topic Pulmonary fibrosis
Myofibroblasts
PGE2
cAMP levels
url https://eprints.nottingham.ac.uk/35925/