FIGURE

H, I

ID

ZDB-FIG-210714-43

Publication

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

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H, I

αKG is a key metabolite responsible for impaired autophagy and proximal tubule dysfunction in cystinotic proximal tubule cells

Relative reactive oxygen species (ROS/mg protein) production in control (CTNS^WT), CRISPR‐generated cystinotic cells (CTNS^−/−) and patient‐derived cystinotic (CTNS^Patient) cells under fed condition (n = 3).

B–D

ROS production (ROS/mg protein) in CTNS^WT, CTNS^−/− and CTNS^Patient cells upon starvation for 4 h in the presence and absence of DMKG (4 h), respectively (n = 3).

Viability test in starved (−AA) CTNS^WT, CTNS^−/− and CTNS^Patient cells in the presence or absence of DMKG (2 mM) for 24 h. Results are shown relative to the fed condition (n = 3).

F, G

Representative confocal micrographs (scale bars are 20 µm) and immunofluorescence analysis of caspase 3/7 activation in DMKG (2 mM)‐treated CTNS^WT, CTNS^−/− and CTNS^Patient cells for 24 h (n = 3).

H, I

Western blotting and densitometric analyses for LC3‐II/LC3‐I ratio in CTNS^WT, CTNS^−/− and CTNS^Patient cells cultured in the presence or in the absence of BafA1 (25 nM) and DMKG (2 mM) for 4 h, respectively (n = 3).

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, D, E, G and I) or Unpaired t‐test (B, C, D, E and G). 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.