Fragiadaki, M. orcid.org/0000-0002-1587-5577, Macleod, F.M. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2020) The controversial role of fibrosis in Autosomal Dominant Polycystic Kidney Disease. International Journal of Molecular Sciences, 21 (23). 8936. ISSN 1661-6596
Abstract
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is characterized by the progressive growth of cysts but it is also accompanied by diffuse tissue scarring or fibrosis. A number of recent studies have been published in this area, yet the role of fibrosis in ADPKD remains controversial. Here, we will discuss the stages of fibrosis progression in ADPKD, and how these compare with other common kidney diseases. We will also provide a detailed overview of some key mechanistic pathways to fibrosis in the polycystic kidney. Specifically, the role of the ‘chronic hypoxia hypothesis’, persistent inflammation, Transforming Growth Factor beta (TGFβ), Janus Kinase/Signal Transducers and Activators of Transcription (JAK/STAT) and microRNAs will be examined. Evidence for and against a pathogenic role of extracellular matrix during ADPKD disease progression will be provided.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
Keywords: | ADPKD; fibrosis; EMT; TGFβ; JAK/STAT; hypoxia; microRNAs; extracellular matrix (ECM); PKD1; PKD2 |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 08 Dec 2020 17:52 |
Last Modified: | 08 Dec 2020 17:52 |
Status: | Published |
Publisher: | MDPI AG |
Refereed: | Yes |
Identification Number: | 10.3390/ijms21238936 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:168786 |