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. Author manuscript; available in PMC: 2021 Jul 18.
Published in final edited form as: Nat Metab. 2021 Jan 18;3(1):33–42. doi: 10.1038/s42255-020-00334-y

Extended Data Fig. 1. MELAS cybrid cells exhibit deficient mitochondrial respiration and undergo apoptosis from nutrient stress.

Extended Data Fig. 1

a. Oxygen Consumption Rates (OCR) measured in control and MELAS cybrid cells. Measurements 4–6 follow the injection of 4 μM oligomycin, measurements 7–9 follow the injection of 4 μM FCCP, and measurements 10–12 follow the injection of 1.5 μM rotenone/4 μM antimycin (n=5 biologically independent samples). b. 48 hour low-glucose survival assay of MELAS cybrid cells treated with the pan-caspase inhibitor Z-VAD-FMK (n=2 biologically independent samples). c. RFLP mapping of ND1 and MELAS mtDNA mutations in cybrids after 24 hour galactose or low-glucose conditions with 1 μM doxycycline. Expected band sizes are 193/159 bp for ND1 and 117/213 bp for MELAS mutations. d–f. BN-PAGE of isolated mitochondria from MELAS cybrids, ND1 cybrids, and Rieske KO fibroblasts treated with 1 μM doxycycline. MELAS cybrids were propagated 24 hours in low-glucose media, ND1 cybrids were propagated for 48 hours in galactose media, and Rieske KO fibroblasts were propagated for 48 hours in high-glucose media. Data are presented as mean values +/− s.e.m. error bars, Student’s t-test with a two-stage linear step-up procedure of Benjamini, Krieger and Yekutieli, with Q = 5%, * q<0.05.