FIGURE

I, J

ID

ZDB-FIG-210714-40

Publication

Jamalpoor et al., 2021 - Cysteamine-bicalutamide combination therapy corrects proximal tubule phenotype in cystinosis

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I, J

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

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).

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).

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.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ EMBO Mol. Med.