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ID

ZDB-FIG-210714-50

Publication

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

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Cysteamine‐bicalutamide combination treatment is safe in patient‐derived cystinotic kidney tubuloids and in wild‐type zebrafish

Patient‐derived cystinotic tubuloids (CTNS^Patient‐1 and CTNS^Patient‐2) and tubuloids established from healthy kidney tissue (CTNS^WT‐1 and CTNS^WT‐2) were differentiated for 7 days and analysed by quantitative real‐time PCR for markers of various segments of the nephron (n = 1).

Brightfield images of cystinotic tubuloids and healthy control tubuloids at the start of treatment and after 5 days of cysteamine (100 μM)‐bicalutamide (35 μM) combination treatment or treatment with medium only (negative control). Scale bars are 2,000 µm.

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Bicalutamide safety screening in cystinotic tubuloids. Tubuloid viability upon treatment with cysteamine (100 μM) in combination with increasing concentrations of bicalutamide was compared to treatment with cysteamine alone (= 100% viability) (per donor n = 4 replicates for each dose).

Survival rates in wild‐type zebrafish upon treatment with bicalutamide (10 µM), and a combination of cysteamine and bicalutamide (1,000 and 10 µM, respectively) (n = 40 embryos per group).

Deformity rates in wild‐type zebrafish upon treatment with bicalutamide (10 µM), and a combination of cysteamine and bicalutamide (1,000 and 10 µM, respectively) (n = 40 embryos per group).

Hatching rates in surviving wild‐type zebrafish evaluated at 72‐ and 96‐h post‐fertilization (hpf) with bicalutamide (10 µM), and a combination of cysteamine and bicalutamide (1,000 and 10 µM, respectively) (n = 40 embryos per group). Drugs were administered at 48‐h post‐fertilization in all experiments dissolved in the swimming water with the specified concentrations.

Data information: Data are expressed as mean ± SEM.

Expression Data

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Antibody Labeling

Phenotype Data

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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.