ZFIN Image: Jamalpoor et al., 2021, I, J

Image

Figure Caption

I, J CRISPR‐generated <italic>CTNS</italic><sup>−/−</sup> ciPTEC demonstrate cystinosis phenotype

A

Quantification of cystine levels (nmol/mg protein) in control (CTNS^WT) (n = 4), CRISPR‐generated cystinotic cells (CTNS^−/−) (n = 6), and patient‐derived cystinotic cells (CTNS^Patient) (n = 4).

B

Quantification of transcription factor EB (TFEB)‐GFP nuclear translocation in CTNS^WT, CTNS^−/−, and CTNS^Patient, respectively. Data are demonstrated as the ratio between number of the cells with nucleus‐TFEB positive over the total number of TFEB‐transfected cells. The ratios were then presented as a fold change compared to control cells (n = 3).

C

TFEB mRNA expression in CTNS^−/− and CTNS^Patient cells compared to control cells (n = 3).

D, E

Representative confocal micrographs and quantification of LC3‐II accumulation in CTNS^WT and CTNS^−/− cells in presence and absence of 25 nM bafilomycin (BafA1) for 4 h, respectively (n = 3). Scale bars are 20 µm.

F–H

Western blotting and densitometric analyses for LC3‐II/LC3‐I ratio and SQSTM1 protein levels in CTNS^WT, CTNS^−/− and CTNS^Patient cells cultured in the presence or in the absence of 25 nM BafA1 for 4 h, respectively (n = 3).

I, J

Representative confocal micrographs and quantification of DQ‐BSA and BSA in CTNS^WT, CTNS^−/− and CTNS^Patient cells, respectively (n = 7–14 quantified images). Scale bars are 20 µm.

Data information: Data are expressed as mean ± SEM. *P‐values < 0.05 were considered to be significant. One‐way ANOVA with Dunnett’s correction (A, B, C, E, G, H, I and J) or Unpaired t‐test (E, G and H). Exact P‐values and statistical tests are listed in Appendix Table S1.

Source data are available online for this figure.

Acknowledgments

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