Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I

BMC Pediatrics

Table 2 The activities of respiratory chain complexes in isolated muscle mitochondria

Patient	Individual respiratory chain complexes activities in isolated muscle mitochondria
	CI/CS		CI + III/CS		CII/CS		CII + III/CS		CIII/ CS		CIV/CS
	patient	age related control range	patient	age related control range	patient	age related control range	patient	age related control range	patient	age related control range	patient	age related control range
1	0.50	0.45–1.05	0.18	0.17–0.31	0.09	0.07–0.27	0.33	0.17–0.47	0.43	0.27–0.85	1.59	1.1–2.22
3	0.13	0.18–0.38	0.08	0.18–0.37	0.04	0.05–0.11	0.24	0.17–0.32	0.67	0.46–0.88	1.21	0.66–2.25
4	0.24	0.45–1.05	0.06	0.17–0.31	0.09	0.07–0.27	0.34	0.17–0.47	0.95	0.27–0.85	0.97	1.1–2.22
5	0.11	0.15–0.41	0.01	0.13–0.25	0.05	0.05–0.11	0.23	0.15–0.27	0.72	0.3–0.56	1.5	0.66–2.25
6	0.06	0.45–1.05	0.08	0.17–0.31	0.07	0.07–0.27	0.26	0.17–0.47	0.72	0.27–0.85	1.12	1.1–2.22
7	0.08	0.18–0.38	0.06	0.18–0.37	0.07	0.05–0.11	0.38	0.17–0.32	0.74	0.46–0.88	1.11	1.16–2.13
8	0.16	0.15–0.41	0.05	0.13–0.25	0.06	0.05–0.11	0.44	0.15–0.27	1.7	0.3–0.56	1.12	0.66–2.25
9	0.28	0.18–0.38	0.03	0.18–0.37	0.09	0.05–0.11	0.38	0.17–0.32	0.66	0.46–0.88	1.27	1.16–2.13
10	0.11	0.18–0.38	0.08	0.18–0.37	0.05	0.05–0.11	0.30	0.17–0.32	0.57	0.46–0.88	1.7	1.16–2.13
11	0.07	0.18–0.38	0.03	0.18–0.37	0.04	0.05–0.11	0.19	0.17–0.32	0.52	0.46–0.88	0.96	1.16–2.13

The reference ranges for individual patients according to age of patients are displayed in next column. Controls are constituted in the three major groups (0–2 years old; 3–18 years old and adults). Alterations in patient’s complexes activities are shown in bold. Abbreviations: CI complex I, NADH coenzyme Q reductase; CI + III complex I + III, NADH cytochrome c reductase; CII complex II, succinate:coenzyme Q reductase; CII + III complex II + III, succinate:cytochrome c oxidoreductase; CIII complex III, coenzyme Q:cytochrome c oxidoreductase; CIV complex IV, cytochrome c oxidase; CS citrate synthase

ISSN: 1471-2431