Table 1.

OXPHOS activities in the patient's fibroblasts. Normal findings.

Oxidoreductases	Patient(mU/mU CS)	Reference values (mU/mU CS)
Complex I	0.55	0.27–0.66
Complex II	0.39	0.34–0.69
Complex III	0.75	0.68–1.49
Complex IV	0.89	0.52–1.32
Complex V	0.22	0.21–0.62
Citrate synthase (CS) (mU/mg protein)	150.00	92–247