Figure 2: Impaired mitochondrial function in myoblasts from MDC1A and Surf1-LS patients.
From: Bioenergetic Impairment in Congenital Muscular Dystrophy Type 1A and Leigh Syndrome Muscle Cells
(A) OCR of control, MDC1A and Surf1-LS myoblasts in response to 5 μM oligomycin, 1 μM FCCP and 1 μM rotenone. For further details see methods section. Decreased oxygen consumption was observed in MDC1A and Surf1-LS myoblasts compared to control cells. (B) Basal respiration; (C) Maximum Respiration; (D) ATP Production; (E) Spare respiration %; (F) Non-mitochondrial respiration; (G) Coupling efficiency. (H) Relative mtDNA content was decreased in MDC1A and Surf1-LS myoblasts compared to control cells. Results are expressed as a fold change of controls. (I) FACS measurement of ΔΨm using JC-1. Results are expressed as % cell population with normal ΔΨm. Columns represent mean values and bars SE. n = 3–10 biological replicates per patient and subject. Statistical significance was assessed by one-way ANOVA followed by Duncan’s post hoc test. p < 0.05 values were considered as statistically significantly different from each other. Letters a, b and c were used to express the differences among groups and columns with the same letter are not significantly different from another.
