Using human urine-derived renal epithelial cells to model kidney disease in inherited ciliopathies

The extreme heterogeneity of renal ciliopathies warrants the use of personalised, patient-specific disease models. Kidney tubular epithelia are exposed to continuous passage of filtrate and viable renal tubular cells are excreted daily in the urine, representing a non-invasive source of patient primary material. These cells can be isolated, cultured and employed for a range of applications, from disease modelling to ex vivo drug testing.