Figure 5 | Scientific Reports

Figure 5

From: Using a quantitative quadruple immunofluorescent assay to diagnose isolated mitochondrial Complex I deficiency

Figure 5

Analysis of Complex I assembly by BN-PAGE. Complex I assembly profiles were analysed using one dimensional blue native polyacrylamide gel electrophoresis (BN-PAGE) (4-16% gradient). Analysis showed a decrease in fully-assembled CI in patients P17, P18, P23 and P24, whilst normal assembly is seen in patients P19, P21, P22 and P25. Complex II was used a loading control. Both OXPHOS complexes were detected by immunoblotting using subunit specific antibodies – NDUFB8 (Complex I) and SDHA (complex II). The original, full length blots are included in the Supplementary Information File (Supplementary Fig. S6).

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