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Pediatric Nephrology
Published in: Pediatric Nephrology 10/2019

01-10-2019 | Educational Review

Fundamental insights into autosomal dominant polycystic kidney disease from human-based cell models

Authors: Caroline Weydert, Jean-Paul Decuypere, Humbert De Smedt, Peter Janssens, Rudi Vennekens, Djalila Mekahli

Published in: Pediatric Nephrology | Issue 10/2019

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Abstract

Several animal- and human-derived models are used in autosomal dominant polycystic kidney disease (ADPKD) research to gain insight in the disease mechanism. However, a consistent correlation between animal and human ADPKD models is lacking. Therefore, established human-derived models are relevant to affirm research results and translate findings into a clinical set-up. In this review, we give an extensive overview of the existing human-based cell models. We discuss their source (urine, nephrectomy and stem cell), immortalisation procedures, genetic engineering, kidney segmental origin and characterisation with nephron segment markers. We summarise the most studied pathways and lessons learned from these different ADPKD models. Finally, we issue recommendations for the derivation of human-derived cell lines and for experimental set-ups with these cell lines.
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Metadata
Title
Fundamental insights into autosomal dominant polycystic kidney disease from human-based cell models
Authors
Caroline Weydert
Jean-Paul Decuypere
Humbert De Smedt
Peter Janssens
Rudi Vennekens
Djalila Mekahli
Publication date
01-10-2019
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 10/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-018-4057-5

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