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. 2017 Apr 17;26(13):2515–2525. doi: 10.1093/hmg/ddx146

Figure 1.

Figure 1

Characterization of mitochondrial function in skeletal muscle. (A) Mitochondrial ATP production rate (MAPR) in fresh skeletal muscle mitochondria, isolated from subject 1, using six different substrate combinations as indicated. Results are presented as the ATP synthesis rate (units) per unit of CS activity (control n=11; age 0–5 years). (B) Respiratory chain enzyme activities of complexes I and III (NADH:cytochrome c reductase), complex I (NADH:coenzyme Q reductase), complexes II and III (succinate:cytochrome c reductase, SCR), complex IV (cytochrome c oxidase) and citrate synthase (CS) were determined. Results are presented as percentage of mean control (n=9; age 0–5 years) values. The range of control values is depicted as ± 2SD. (C) COX/SDH double staining of fresh-frozen skeletal muscle from subject 1. (D) MAPR as in (A) from subject 2. (E) Respiratory chain enzyme activities as in (B) from subject 2. (F) COX/SDH double staining of fresh-frozen skeletal muscle from subject 2. (G) Gomori-trichrome staining of fresh-frozen skeletal muscle from subject 2 revealing ragged-red fibres (RRF). (H) Electropherogram of POLγA from subject 1, 2 and control.