FIGURE

G

ID

ZDB-FIG-210714-46

Publication

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

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Cysteamine–bicalutamide combination treatment efficiently lowers lysosomal cystine, abolishes αKG‐mediated autophagy distortion and cell death in cystinotic proximal tubule cells

Cell viability test of DMKG (2 mM)‐treated CRISPR‐generated cystinotic cells (CTNS^−/−) upon pre‐treatment with cysteamine (100 µM), bicalutamide (35 µM), and a combination of cysteamine and bicalutamide (100 and 35 µM, respectively). Results are shown relative to the starved condition (n = 3).

Western blotting and densitometric analyses for LC3‐II/LC3‐I ratio in DMKG (2 mM)‐treated CTNS^WT and CTNS^−/− cells upon pre‐treatment with cysteamine, bicalutamide, and a combination of cysteamine and bicalutamide (n = 3).

Relative reactive oxygen species (ROS/mg protein) production in DMKG‐treated CTNS^WT and CTNS^−/− cells upon pre‐treatment with cysteamine, bicalutamide, and a combination of cysteamine and bicalutamide (n = 3).

D, E

Representative confocal micrographs and quantification of DQ‐BSA in CTNS^WT, and CTNS^−/− cells upon treatment with bicalutamide. Scale bars are 20 µm (n = 8–14 quantified images).

Quantification of cystine levels (nmol/mg protein) by HPLC‐MS/MS in CTNS^−/− in the absence of the drug (NT) (n = 4) or upon treatment with cysteamine (n = 6), bicalutamide (n = 6), and a combination of cysteamine and bicalutamide (n = 6).

Quantification of cystine levels (nmol/mg protein) by HPLC‐MS/MS in CTNS^Patient in the absence of the drug (NT) (n = 4) or upon treatment with cysteamine (n = 4), bicalutamide (n = 4), and a combination of cysteamine and bicalutamide (n = 6).

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, F and G) or unpaired t‐test (B, C, E, F 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.