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. Author manuscript; available in PMC: 2020 Jul 1.
Published in final edited form as: Semin Cell Dev Biol. 2018 Sep 20;91:153–168. doi: 10.1016/j.semcdb.2018.08.015

Figure 4. Application of hPSC-derived kidney tissues for disease modelling.

Figure 4.

A. Outline for the generation of patient-derived and isogenic iPSC lines from a Maizner-Saldino syndrome patient carrying compound heterozygote mutations in the IFT140 gene [107]. The protocol for simultaneous generation of patient and gene corrected lines is described in Howden et al [79]. B. Approach to the characterisation of disease related changes using cells isolated from hPSC-derived kidney organoids [107]. C. Immunofluorescence imaging of renal tubular epithelial cells within iPSC-derived kidney organoids showing the presence of clubbed cilia in patient organoids and wildtype cilia in organoids from gene corrected lines. D. Bright field images of intact glomeruli isolated by sieving dissociated hPSC-derived kidney organoids at low and high power. Figure courtesy of Dr. Lorna Hale.