New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease is the most common inherited kidney disease and results from mutations in the polycystin 1 gene (PKD1) or the polycystin 2 gene (PKD2). The disease is characterised by the progressive development of fluid-filled cysts derived from renal tubular epithelial...

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Main Authors: Maurizio Salvadori, Aris Tsalouchos
Format: Article
Language:English
Published: European Medical Journal 2017-07-01
Series:European Medical Journal Nephrology
Subjects:
Online Access:https://www.emjreviews.com/nephrology/article/new-therapies-targeting-cystogenesis-in-autosomal-polycystic-kidney-disease/
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author Maurizio Salvadori
Aris Tsalouchos
author_facet Maurizio Salvadori
Aris Tsalouchos
author_sort Maurizio Salvadori
collection DOAJ
description Autosomal dominant polycystic kidney disease is the most common inherited kidney disease and results from mutations in the polycystin 1 gene (PKD1) or the polycystin 2 gene (PKD2). The disease is characterised by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that destroy the architecture of the renal parenchyma and lead to kidney failure. Until recently, the causes and the molecular pathways that lead to cystogenesis remained obscure. In the last decade, enormous progress has been made in understanding the pathogenesis of autosomal dominant polycystic kidney disease and developing new therapies. The purpose of this review is to provide an update on the promising therapies that are being developed and tested, based on knowledge of recent advances in molecular and cellular targets involved in cystogenesis.
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spelling doaj.art-4c4dc31103ea48ea8840da53985c4bf12022-12-22T00:21:44ZengEuropean Medical JournalEuropean Medical Journal Nephrology2053-42482017-07-0151102111New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney DiseaseMaurizio Salvadori0Aris Tsalouchos1Renal Unit, Careggi University Hospital, Florence, ItalyDivision of Nephrology, Careggi University Hospital, Florence, ItalyAutosomal dominant polycystic kidney disease is the most common inherited kidney disease and results from mutations in the polycystin 1 gene (PKD1) or the polycystin 2 gene (PKD2). The disease is characterised by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that destroy the architecture of the renal parenchyma and lead to kidney failure. Until recently, the causes and the molecular pathways that lead to cystogenesis remained obscure. In the last decade, enormous progress has been made in understanding the pathogenesis of autosomal dominant polycystic kidney disease and developing new therapies. The purpose of this review is to provide an update on the promising therapies that are being developed and tested, based on knowledge of recent advances in molecular and cellular targets involved in cystogenesis.https://www.emjreviews.com/nephrology/article/new-therapies-targeting-cystogenesis-in-autosomal-polycystic-kidney-disease/Adult autosomal polycystic kidney disease (ADPKD)cystogenesisvasopressin 2 receptorssomatostatin analoguesmammalian target of rapamycin (mTOR) signalling.
spellingShingle Maurizio Salvadori
Aris Tsalouchos
New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
European Medical Journal Nephrology
Adult autosomal polycystic kidney disease (ADPKD)
cystogenesis
vasopressin 2 receptors
somatostatin analogues
mammalian target of rapamycin (mTOR) signalling.
title New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
title_full New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
title_fullStr New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
title_full_unstemmed New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
title_short New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
title_sort new therapies targeting cystogenesis in autosomal polycystic kidney disease
topic Adult autosomal polycystic kidney disease (ADPKD)
cystogenesis
vasopressin 2 receptors
somatostatin analogues
mammalian target of rapamycin (mTOR) signalling.
url https://www.emjreviews.com/nephrology/article/new-therapies-targeting-cystogenesis-in-autosomal-polycystic-kidney-disease/
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