The European Rare Kidney Disease Reference Network


Research Project

Project Title:

Neocyst WP5: Epithelial function in cystic kidney disease: defects of cell adhesion and epithelial morphogenesis

Project Type:

Translational research

Disease group(s):

Renal malformations

Project Summary:

The NEOCYST project 5 is dedicated to translational research on cell biological questions. Biological samples of genotyped and deeply phenotyped patients are employed to elucidate molecular mechanisms that are relevant for the progression of cystic kidney diseases and that can explain the partial phenotypic overlap between different cystic kidney disease entities.
In Hannover, the project focuses on altered epithelial cell characteristics that lead to defective homeostasis of tubular and collecting duct epithelia and that cause or deteriorate renal cyst formation. Altered cell properties are being studied ex vivo using urine-derived renal epithelial cells (UREC) and correlated to quantitative parameters of cell adhesion and epithelial morphogenesis. The study uses ARPKD as a model to determine relevant parameters, and subsequently, will include all cystic kidney diseases studied by the NEOCYST consortium. Assessment of quantitative cell characteristics based on state-of-the-art cell biological tools is expected to reveal disease-specific alterations of cell function. Correction of epithelial cell defects will be addressed by ex vivo studies of UREC from patients and may ultimately allow identification of potential novel therapeutic approaches for cystic kidney disease and for individualized treatment.

Lead principal investigator(s):

Dieter Haffner, Hannover
Wolfgang Ziegler, Hannover

Project Period:

04/2016   -   02/2019


National funding agency

Project web page:

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