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Fig. 3 | BMC Nephrology

Fig. 3

From: Use of patient derived urine renal epithelial cells to confirm pathogenicity of PKHD1 alleles

Fig. 3

ARPKD URECs display an elongated ciliary phenotype. a Representative immunofluorescence images of wild type control URECs and ARPKD URECs, serum starved for 48 h. ARPKD cells display elongated cilia when compared to control, with swellings along the axoneme and at the ciliary tip. Green – ARL13B, Red – acetylated α-tubulin. Scale bar 5 μm. b Quantification of cilia length in control URECs and ARPKD URECs. The mean cilia length measured for control URECs was 5.5 μm. Mean length of ARPKD URECs was 6.5 μm. *P < 0.001, unpaired t test, n = 338. c Quantification of the percentage of cilia that display a bulbous phenotype with one or more blebs along the axoneme in control and ARPKD URECs. The percentage of cilia with blebs was 25% in control cells and 69% in ARPKD URECs. *P < 0.001, Fisher’s exact test, n = 229. d Quantification of percentage of ciliated cells in control URECs and ARPKD URECs. The percentage of ciliated cells for control cells was 61%. The percentage of ciliated cells measured for ARPKD cells was 48%. *P < 0.001, Fisher’s exact test, n = 694

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