Fig. 2

UREC preparations from patients with hereditary cystic kidney diseases and controls, yield and properties in 2D culture. A Scheme of UREC culture based on cell harvest from 30 to 50 ml of spontaneous urine and establishment of renal tubular epithelial cell colonies. B Yield of independent UREC preparations after 13–15 days in culture, as indicated by filled circles, is highly variable and most efficient in ARPKD and NPH cohorts. Repeat preparations for patients are included. Note low outcome for controls (filled triangles), single children (and pools) with no cystic kidney genetics and normal organ function. Median values of cell count are indicated. (Kruskal–Wallis, Dunn’s; */*** for p < 0.05/p < 0.001). C Population doubling time in 2D culture of spare to medium dense colonies was determined based on metabolic activity of cells growing in medium with 0.5% serum. Median times of cell doubling are indicated and no significant differences observed (Kruskal–Wallis). D Representative UREC cultures showing formation of cell–cell junctions in dense 2D culture, as stained for E-cadherin (red, a–c) and for zonula occludens 1 (ZO-1; green, a′–c′) in corresponding micrographs. There was individual variability in signal strength but no evidence observed for defects in formation of adherens or tight junctions in any of the preparations. Size bar 20 µm