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. 2015 Mar 27;128(12):895–904. doi: 10.1042/CS20140705

Table 1. Peak exercise parameters, 31P-MRS examination on calf evaluating in vivo mitochondrial function and assessment of respiratory chain complex activities in skeletal muscle homogenates.

BPM, beats per min; DCPIP, 2,6-dichlorophenol-indophenol; n/a, not available; t1/2 PCR (s), half time for PCR recovery from end exercise to baseline concentrations; VO2, oxygen uptake. Enzyme activities are expressed as nmol of NADH oxidized/min per unit of citrate synthase (CS) for CI, nmol of DCPIP reduced/min per unit of CS for CII (succinate:ubiquinone-1 reductase) and the apparent first-order rate constant/s per unit of CS for CIII and CIV (×103).

Parameter Patient 1 Patient 2 Reference data (means ± S.D.)
CPET
Peak exercise data Absolute value (% of predicted) Absolute value (% of predicted) n/a
Heart rate (BPM) 172 (89) 180 (92) n/a
VO2 (ml/min) 0.575 (27) 0.638 (22) n/a
VO2 (ml/min per kg) 8 (27) 14 (22) n/a
VO2 (% of predicted VO2) 27 22 n/a
Power (W) 35 (21) 53 (27) n/a
AT
ATVO2 (ml/min per kg) 6 10 n/a
ATVO2 (% of predicted peak VO2) 20 16 n/a
ATVO2 (% of recorded peak VO2) 75 74 n/a
31P-MRS [14]
End exercise data
t1/2 PCr 52.5 n/a 29.2 ± 3.1
t1/2 ADP 31.4 n/a 20 ± 1
Respiratory chain complex activities Biopsy 1 Biopsy 2 Biopsy Controls (n=25)
CI/CS 0.018 0.009 0.001 0.104 ± 0.036
CII/CS 0.092 0.127 0.070 0.145 ± 0.047
CIII/CS 0.673 1.790 0.634 0.554 ± 0.345
CIV/CS 0.834 1.394 1.216 1.124 ± 0.511