The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease

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 w...

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Main Authors:	Maria Fragiadaki, Fiona M. Macleod, Albert C. M. Ong
Format:	Article
Language:	English
Published:	MDPI AG 2020-11-01
Series:	International Journal of Molecular Sciences
Subjects:	ADPKD fibrosis EMT TGFβ JAK/STAT hypoxia
Online Access:	https://www.mdpi.com/1422-0067/21/23/8936

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spelling	doaj-378e396ab21e4fe99d60004c487c403a2020-11-27T08:02:41ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-11-01218936893610.3390/ijms21238936The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney DiseaseMaria Fragiadaki0Fiona M. Macleod1Albert C. M. Ong2Department of Infection, Immunity and Cardiovascular Disease, Faculty of Medicine, Dentistry & Health, University of Sheffield, Sheffield S10 2RX, UKDepartment of Infection, Immunity and Cardiovascular Disease, Faculty of Medicine, Dentistry & Health, University of Sheffield, Sheffield S10 2RX, UKDepartment of Infection, Immunity and Cardiovascular Disease, Faculty of Medicine, Dentistry & Health, University of Sheffield, Sheffield S10 2RX, UKAutosomal 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.https://www.mdpi.com/1422-0067/21/23/8936ADPKDfibrosisEMTTGFβJAK/STAThypoxia
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Maria Fragiadaki Fiona M. Macleod Albert C. M. Ong
spellingShingle	Maria Fragiadaki Fiona M. Macleod Albert C. M. Ong The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease International Journal of Molecular Sciences ADPKD fibrosis EMT TGFβ JAK/STAT hypoxia
author_facet	Maria Fragiadaki Fiona M. Macleod Albert C. M. Ong
author_sort	Maria Fragiadaki
title	The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
title_short	The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
title_full	The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
title_fullStr	The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
title_full_unstemmed	The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
title_sort	controversial role of fibrosis in autosomal dominant polycystic kidney disease
publisher	MDPI AG
series	International Journal of Molecular Sciences
issn	1661-6596 1422-0067
publishDate	2020-11-01
description	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.
topic	ADPKD fibrosis EMT TGFβ JAK/STAT hypoxia
url	https://www.mdpi.com/1422-0067/21/23/8936
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The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease

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