Fig. 6

Western blot analysis reveals comparable levels of mitochondria-associated proteins between healthy and POLG^Q811R variant MDNS. a Abundance levels for mitochondrial markers MFN1, MFN2 and OPA1 involved in mitochondrial fusion, and MFF, FIS1 and MIEF1 regulating mitochondrial fission in POLG1 variant and healthy control MDNS, identified by LC MS/MS quantitative proteomics. b Western blot analysis confirms no difference in expression levels of mitochondrial transcription factor TFAM, fusion-related OPA1 and MFN2 and fission-related DRP1 in POLG1 variant and healthy control MDNS. c Quantification of protein levels from (b), normalized to b-ACTIN. Results are presented as ± SEM; n = 4 independent experiments; unpaired t-test