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

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...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2019-10, Vol.34 (10), p.1697-1715
Main Authors: Weydert, Caroline, Decuypere, Jean-Paul, De Smedt, Humbert, Janssens, Peter, Vennekens, Rudi, Mekahli, Djalila
Format: Article
Language:English
Subjects:
Animal models
Cell culture
Cell lines
Diagnosis
Educational Review
Gene mutations
Genetic engineering
Kidney diseases
Kidneys
Medicine
Medicine & Public Health
Nephrectomy
Nephrology
Pediatrics
Polycystic kidney
Polycystic kidney disease
Risk factors
Stem cells
Urine
Urology
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Summary: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.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-018-4057-5