Fig. 5.

Mitochondrial membrane potential (Δψm) is lower in cystinotic cells and in HK-2 cells silenced for CTNS gene and rescued by cysteamine or 8Br-cAMP treatment. a Analysis of mitochondrial potential (Δψm) in ciPTEC obtained from a healthy volunteer (CTNS^+/+) and from two cystinotic patients (delCTNS^−/− or mutCTNS^−/−) treated with 100 µM cysteamine (MEA) or 100 µM 8Br-cAMP for 24 h. b Analysis of mitochondrial potential (Δψm) in HK-2 cells transfected with siGENOME human CTNSgene (siCTNS) or non-targeting siRNA (Mock) and treated with 100 µM cysteamine (MEA) or 100 µM 8Br-cAMP for 24 h. Data result from the analysis of the fluorescence intensity mean expressed in arbitrary units (A.U.) ± SEM measured in at least 30 cells (a) and in about 100 cells (b) (Student’s t test)