Fig. 3.

Several subunits of complex I and complex V are reduced in cystinotic ciPTEC cells and restored by cysteamine treatment. a Immunoblotting analysis of ciPTEC obtained from a healthy volunteer (CTNS^+/+) and cystinotic patient (delCTNS^−/−). b Immunoblotting analysis of CTNS^+/+ and cystinotic patient (mutCTNS^−/−). a, b Where indicated, the cells were treated with 100 µM cysteamine (MEA) or DMSO (vehicle) for 24 h. After incubation, proteins of cellular lysate were loaded on 8% SDS-PAGE, transferred to nitrocellulose membranes, and immunoblotted with the antibodies described in the figure. Protein loading was assessed by reprobing the blots with the actin antibody. Densitometric analysis was performed by VersaDoc imaging system BioRad, using Quantity One software. The histograms represent the means values ± SEM of the ratio of ADU (arbitrary densitometric units) of the immuno-revealed protein normalized on actin level (Student’s t test)