SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis

Fibrosis and organ failure is a common endpoint for many chronic liver diseases. Much is known about the upstream inflammatory mechanisms provoking fibrosis and downstream potential for tissue remodeling. However, less is known about the transcriptional regulation in vivo governing fibrotic matrix d...

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Main Authors: Athwal, Varinder S., Pritchett, James, Llewellyn, Jessica, Martin, Katherine, Camacho, Elizabeth, Raza, Sayyid M.A., Phythian-Adams, Alexander, Birchall, Lindsay J., Mullan, Aoibheann F., Su, Kim, Pearmain, Laurence, Dolman, Grace, Zaitoun, Abed M., Friedman, Scott L., MacDonald, Andrew, Irving, William L., Guha, Indra N., Hanley, Neil A., Hanley, Karen Piper
Format: Article
Published: Wiley Open Access 2017
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Online Access:https://eprints.nottingham.ac.uk/46609/
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author Athwal, Varinder S.
Pritchett, James
Llewellyn, Jessica
Martin, Katherine
Camacho, Elizabeth
Raza, Sayyid M.A.
Phythian-Adams, Alexander
Birchall, Lindsay J.
Mullan, Aoibheann F.
Su, Kim
Pearmain, Laurence
Dolman, Grace
Zaitoun, Abed M.
Friedman, Scott L.
MacDonald, Andrew
Irving, William L.
Guha, Indra N.
Hanley, Neil A.
Hanley, Karen Piper
author_facet Athwal, Varinder S.
Pritchett, James
Llewellyn, Jessica
Martin, Katherine
Camacho, Elizabeth
Raza, Sayyid M.A.
Phythian-Adams, Alexander
Birchall, Lindsay J.
Mullan, Aoibheann F.
Su, Kim
Pearmain, Laurence
Dolman, Grace
Zaitoun, Abed M.
Friedman, Scott L.
MacDonald, Andrew
Irving, William L.
Guha, Indra N.
Hanley, Neil A.
Hanley, Karen Piper
author_sort Athwal, Varinder S.
building Nottingham Research Data Repository
collection Online Access
description Fibrosis and organ failure is a common endpoint for many chronic liver diseases. Much is known about the upstream inflammatory mechanisms provoking fibrosis and downstream potential for tissue remodeling. However, less is known about the transcriptional regulation in vivo governing fibrotic matrix deposition by liver myofibroblasts. This gap in understanding has hampered molecular predictions of disease severity and clinical progression and restricted targets for antifibrotic drug development. In this study we show the prevalence of SOX9 in biopsies from patients with chronic liver disease correlated with fibrosis severity and accurately predicted disease progression towards cirrhosis. Inactivation of Sox9 in mice protected against both parenchymal and biliary fibrosis, improved liver function and ameliorated chronic inflammation. SOX9 was downstream of mechanosignaling factor, YAP1. These data demonstrate a role for SOX9 in liver fibrosis and open the way for the transcription factor and its dependent pathways as new diagnostic, prognostic and therapeutic targets in patients with liver fibrosis.
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spelling nottingham-466092020-05-04T19:16:10Z https://eprints.nottingham.ac.uk/46609/ SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis Athwal, Varinder S. Pritchett, James Llewellyn, Jessica Martin, Katherine Camacho, Elizabeth Raza, Sayyid M.A. Phythian-Adams, Alexander Birchall, Lindsay J. Mullan, Aoibheann F. Su, Kim Pearmain, Laurence Dolman, Grace Zaitoun, Abed M. Friedman, Scott L. MacDonald, Andrew Irving, William L. Guha, Indra N. Hanley, Neil A. Hanley, Karen Piper Fibrosis and organ failure is a common endpoint for many chronic liver diseases. Much is known about the upstream inflammatory mechanisms provoking fibrosis and downstream potential for tissue remodeling. However, less is known about the transcriptional regulation in vivo governing fibrotic matrix deposition by liver myofibroblasts. This gap in understanding has hampered molecular predictions of disease severity and clinical progression and restricted targets for antifibrotic drug development. In this study we show the prevalence of SOX9 in biopsies from patients with chronic liver disease correlated with fibrosis severity and accurately predicted disease progression towards cirrhosis. Inactivation of Sox9 in mice protected against both parenchymal and biliary fibrosis, improved liver function and ameliorated chronic inflammation. SOX9 was downstream of mechanosignaling factor, YAP1. These data demonstrate a role for SOX9 in liver fibrosis and open the way for the transcription factor and its dependent pathways as new diagnostic, prognostic and therapeutic targets in patients with liver fibrosis. Wiley Open Access 2017-11-06 Article PeerReviewed Athwal, Varinder S., Pritchett, James, Llewellyn, Jessica, Martin, Katherine, Camacho, Elizabeth, Raza, Sayyid M.A., Phythian-Adams, Alexander, Birchall, Lindsay J., Mullan, Aoibheann F., Su, Kim, Pearmain, Laurence, Dolman, Grace, Zaitoun, Abed M., Friedman, Scott L., MacDonald, Andrew, Irving, William L., Guha, Indra N., Hanley, Neil A. and Hanley, Karen Piper (2017) SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis. EMBO Molecular Medicine, 9 (12). pp. 1696-1710. ISSN 1757-4684 SOX9; YAP1; Liver fibrosis; Extracellular matrix; Hepatic stellate cells http://embomolmed.embopress.org/content/early/2017/11/06/emmm.201707860 doi:10.15252/emmm.201707860 doi:10.15252/emmm.201707860
spellingShingle SOX9; YAP1; Liver fibrosis; Extracellular matrix; Hepatic stellate cells
Athwal, Varinder S.
Pritchett, James
Llewellyn, Jessica
Martin, Katherine
Camacho, Elizabeth
Raza, Sayyid M.A.
Phythian-Adams, Alexander
Birchall, Lindsay J.
Mullan, Aoibheann F.
Su, Kim
Pearmain, Laurence
Dolman, Grace
Zaitoun, Abed M.
Friedman, Scott L.
MacDonald, Andrew
Irving, William L.
Guha, Indra N.
Hanley, Neil A.
Hanley, Karen Piper
SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title_full SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title_fullStr SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title_full_unstemmed SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title_short SOX9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
title_sort sox9 predicts progression towards cirrhosis in patients while its loss protects against liver fibrosis
topic SOX9; YAP1; Liver fibrosis; Extracellular matrix; Hepatic stellate cells
url https://eprints.nottingham.ac.uk/46609/
https://eprints.nottingham.ac.uk/46609/
https://eprints.nottingham.ac.uk/46609/