Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that results from mutations in PKD1 or PKD2. The disease is characterized by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that destroy the architecture of...

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Main Authors: Maurizio Salvadori, Aris Tsalouchos
Format: Article
Language:English
Published: Codon Publications 2017-03-01
Series:Journal of Renal and Hepatic Disorders
Subjects:
Online Access:https://jrenhep.com/index.php/jrenhep/article/view/10
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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 (ADPKD) is the most common inherited kidney disease that results from mutations in PKD1 or PKD2. The disease is characterized 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 ADPKD and the development of new therapies. The purpose of this review is to 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-e3de624b0ab4435ca79eae2a527e65112022-12-22T01:47:43ZengCodon PublicationsJournal of Renal and Hepatic Disorders2207-37442017-03-011110.15586/jrenhep.2017.107Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney DiseaseMaurizio Salvadori0Aris Tsalouchos1Careggi University Hospital, Viale Pieraccini, 18, 50139, Florence, ItalyDivision of Nephrology, Azienda Ospedaliera Careggi, Largo Alessandro Brambilla, 3, 50134 ,Florence, ItalyAutosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that results from mutations in PKD1 or PKD2. The disease is characterized 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 ADPKD and the development of new therapies. The purpose of this review is to 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://jrenhep.com/index.php/jrenhep/article/view/10adult autosomal polycystic kidney diseasecystogenesismTOR signallingsomatostatin analoguesvasopressin 2 receptor
spellingShingle Maurizio Salvadori
Aris Tsalouchos
Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
Journal of Renal and Hepatic Disorders
adult autosomal polycystic kidney disease
cystogenesis
mTOR signalling
somatostatin analogues
vasopressin 2 receptor
title Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
title_full Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
title_fullStr Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
title_full_unstemmed Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
title_short Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease
title_sort novel therapeutic strategies targeting molecular pathways of cystogenesis in autosomal polycystic kidney disease
topic adult autosomal polycystic kidney disease
cystogenesis
mTOR signalling
somatostatin analogues
vasopressin 2 receptor
url https://jrenhep.com/index.php/jrenhep/article/view/10
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