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Kidney Week

Abstract: TH-PO654

Tubular Epithelial Cell Function in Polycystic Kidney Disease (PKD): Analysis of Cells from Children with ARPKD and NPH

Session Information

  • Pediatric Nephrology
    November 02, 2017 | Location: Hall H, Morial Convention Center
    Abstract Time: 10:00 AM - 10:00 AM

Category: Developmental Biology and Inherited Kidney Diseases

  • 403 Pediatric Nephrology


  • Ziegler, Wolfgang H., Hannover Medical School, Hannover, Germany
  • Soetje, Birga, Hannover Medical School, Hannover, Germany
  • Swolana, Kathrin, Hannover Medical School, Hannover, Germany
  • Mertens, Arne, Hannover Medical School, Hannover, Germany
  • Khera, Amrit, Hannover Medical School, Hannover, Germany
  • Haffner, Dieter, Hannover Medical School, Hannover, Germany

PKD-related cellular defects are best understood genetically or in epithelial (cell) models analysing cell proliferation and signalling. Use of primary patient-derived renal epithelial cells provides a unique opportunity to study and quantify cell properties in epithelial function-related assays. Apart from the analysis of differences due to specific mutation, it appears interesting to correlate the severity of the renal disease i.e. GFR or organ size / morphology with alterations in epithelial cell function determined ex vivo.


We culture urine-derived renal epithelial cells (URECs) of patients with genetically confirmed causes of PKD, autosomal recessive polycystic kidney disease (ARPKD) and nephronophtisis (NPH), and of age-matched healthy controls. Populations of primary cells obtained within 14 days of culture are tested with respect to their proliferation rates, formation of cell-cell junctions in monolayer and barrier function (impedance) in 2D culture. In addition, their capacity to build spheroids and form cilia is addressed in 3D culture conditions using matrigel and micro-patterned adhesion chips.


URECs from cohorts of patients (5-6 each) with ARPKD or NPH (mostly NPHP-1 mutation), and controls are compared in cell culture to measure quantitative characteristics that can be correlated to clinical parameters and progress of PKD. We observe a much higher success rate of epithelial cell cultivation from urine of PKD patients. Cells are mostly of collecting duct origin as determined by aquaporin 2- positive staining. MTS-based assessment of cell proliferation shows relatively stable rates between days 10 and 15 (20) of culture, which are moderately higher in patient cells. Analysis of spheroid formation in matrigel (6 days) reveals on average bigger clusters of patient cells and an individual tendency of defective lumen formation. Barrier function of UREC monolayers is increased in a patient-specific manner.


Determination of genotype and / or disease state specific renal epithelial cell properties in URECs is expected to provide a better understanding of the mechanism and progression of disease processes in renal epithelium and may provide options for testing of pharmaceutical intervention.


  • Private Foundation Support