Fig 1: Protein analysis in six patients with heteroplasmic mutations in MT-ND5 gene. a Comparison of steady-state levels of several OXPHOS-related proteins in P5, P6, P7, P9 and P10 in fibroblasts using SDS-PAGE/WB. As a control was used primary dermal fibroblasts (ATCC® PCS-201-010™), 50 and 100% demonstrate loading dose of protein amount. b BN-PAGE/WB of OXPHOS complexes in isolated mitochondria from the muscle of P8. As a control was used human muscle mitochondria from heathy adult, 25; 50 and 100% demonstrate loading dose of protein amount. c The quantification of western blot signals from A by densitometric analysis. Relative signals intensity of individual OXPHOS antibodies were normalized to intensity of loading control β-tubulin. d The quantification of western blot signals from B by densitometric analysis