Figure 3.

The BHD-derived renal cancer cell line UOK257 shows abnormal ciliation. (A) UOK257 and UOK257-2 cells were maintained at confluence for indicated amount of days, fixed and stained with acetylated alpha tubulin to mark the ciliary axoneme and pericentrin to mark the ciliary base. Nuclei were stained with DAPI and the percentage of ciliated cells was counted using ImageJ. After 6 days of confluence, UOK257 had a significantly reduced number of cilia compared with UOK257-2 (P = 0.023). After 10 days of confluence, both cell lines have a similar number of cilia (P = 0.422). Error bars represent standard error of the mean. A minimum of 365 cells were counted per cell line per time point. *P < 0.05. (B) Sequencing of genomic DNA isolated from HK-2 and UOK257 cells showing the presence of the wild-type allele (starting sequence indicated by the arrow).