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Background and Aims In hereditary PKD, epithelial cell defects are mostly caused by mutation of proteins of the cilia-centrosome complex. Use of primary cells from patients allows to study specific cell characteristics and altered activation of signal pathways in epithelial cell function-related culture models. In this ex vivo approach, comparison of cell properties is based on defined genetic origin and can be related to kidney function at the time of UREC collection, revealing consequences of genetic disposition and kidney-stress related changes on cell function. Method The study considers patients with genetically confirmed causes of PKD, autosomal recessive polycystic kidney disease (ARPKD), nephronophtisis (NPH), Bardet-Biedl syndrome (BBS), and age-matched controls with normal kidney function. UREC are grown from cellular urine sediment using selective growth conditions. Primary cells are cultured up-to 21 days (passages 2 & 3) and tested with respect to their proliferation, tubular origin and epithelial properties in 2D/3D culture conditions. To allow pathway analysis and improve control conditions, immortalized UREC lines are also included. Results UREC preparations of cohorts from ARPKD, NPH, and BBS patients, and controls were studied quantitatively to determine specific epithelial cell properties. Considering basic characteristics of each primary culture including cell morphology, expression of epithelial markers, and formation of cell-cell adhesions, the potential of UREC cells to polarize and generate liquid-filled epithelial spheroids was measured. The capacity of lumen formation in 3D culture strongly varies among individual cell preparations and appears to reflect the genetic origin of UREC cells, whereas no correlation to (remaining) kidney function (eGFR) is observed. Conclusion Determining specific, quantitative UREC cell properties related to genetics and kidney function, we expect to gain a better mechanistic understanding of cellular and renal epithelial defects in PKD patients. Ex vivo analysis based on UREC cells may provide options for testing of personalized pharmaceutical intervention.