Research Paper Volume 14, Issue 4 pp 1651—1664

Mitochondrial dysfunction in mandibular hypoplasia, deafness and progeroid features with concomitant lipodystrophy (MDPL) patients


Figure 3. Functional mitochondrial evaluation of MDPL and WT HDFs. Flow cytometry quantification of (A) total reactive oxygen species using CM-H2DCFDA in HDFs WT and MDPL ones. (B) Flow cytometry quantification of mitochondrial superoxide using MitoSOX Red in HDFs WT and in MDPL ones. The error bar indicated in panels A and B is the average of two independent experiments. (C) Confocal analysis following SOD2 immunofluorescence and its quantification (**p < 0.01). (D) JC1 staining for flow cytometry to evaluate changes in mitochondrial potential membrane and the quantification of red/green fluorescence intensity ratio.