Table 1

Mitochondrial respiratory chain activities

AssaysPatientsReference value
Flux experiments
 Complexes I and III
 Pyruvate + malate cytochrome oxidase0.2450.3270.4260.1640.4180.1390.1–1.0
 Glutamate cytochrome reductase0.0090.0190.0980.1470.2620.0250.1–1.0
 Oxoglutarate cytochrome reductase0.2960.1640.4260.1150.3030.1150.1–1.0
Complexes II and III
 Succinate cytochrome reductase0.4750.1390.6720.4090.4090.2540.1–6.0
Complex IV
 Cytochrome oxidase0.3950.2990.9010.6550.9840.590.1–5.0
Specific assays
Complex I0.8360.4090.8610.0910.1–1.0
Complex II4.9189.83510.3591.9673.0–15.0
  • Respiratory chain activities expressed as μmol/g/min (rate constant k/g/min for COX). Complexes I and III are specific assays. Patients 4, 14, 30, and 32 have been previously reported.26