Table 2. Activities of mitochondrial respiratory chain enzymes in fibroblasts.

Enzyme	Specific activity in patient's cellsnmol/min/mg protein (% of mean)	Normal rangesnmol/min/mg protein
ETC Complex I	499 (83)	602+/−316 (276–806)
ETC Complex I+III (Total)Rotenone sensitive	249 (81)76.5 (76)	307+/−120 (170–410)100+/−40 (50–136)
ETC Complex II	5.84 (77)	7.6+/−5.9 (2.1–13.1)
ETC Complex II+III	13.4 (65)	20.6+/−11.7 (8.2–28.6)
ETC Complex IV	23.3 (89)	26.2+/−17.5 (8.2–42.4)
Citrate synthase (control)	13.9	(12.9–60.6)