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Pediatric Nephrology

Erschienen in:

13.09.2018 | Educational Review

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

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

Erschienen in: Pediatric Nephrology | Ausgabe 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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Titel: Fundamental insights into autosomal dominant polycystic kidney disease from human-based cell models
verfasst von: Caroline Weydert
Jean-Paul Decuypere
Humbert De Smedt
Peter Janssens
Rudi Vennekens
Djalila Mekahli
Publikationsdatum: 13.09.2018
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 10/2019
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-018-4057-5

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

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