Table 1.
Respiratory chain enzyme activities in multiple tissues
| Tissue/complex | Activity� (controls) | SD | Ratio/CS� (controls) | SD | Ratio/complex II� (controls) | SD |
|---|---|---|---|---|---|---|
| MUSCLE | ||||||
| Complex I | 5�.0� (23�.6–74�.8) | −6�.0 | 37� (98–271) | −6�.5 | 67� (285–767) | −7�.2 |
| Complex II | 74�.2� (49�.0–133�.4) | −0�.4 | 550� (251–573) | 1�.4 | NA | NA |
| Complex III | 16�.1� (5�.7–31�.4) | 0�.2 | 119� (19–172) | 0�.7 | 216� (45–369) | 0�.3 |
| Complex II–III | 101�.6� (34�.2–107�.6) | 1�.4 | 753� (172–472) | 0�.3 | 1370� (549–1226) | 2�.1 |
| Complex IV | 1�.5� (1�.1–3�.8) | −1�.1 | 11� (4–23) | −1�.0 | 20� (11–68) | −1�.1 |
| Citrate synthase | 134�.9� (159�.8–353�.3) | −2�.3 | NA | NA | NA | NA |
| HEART | ||||||
| Complex I | 5�.9� (146�.9–366�.9) | −11�.6 | 5� (144–376) | −12�.5 | 14� (506–1064) | −13�.7 |
| Complex II | 431�.7� (176�.4–412�.5) | 1�.4 | 387� (235–356) | 2�.3 | NA | NA |
| Complex III | 208�.7� (16�.5–260�.4) | 1�.3 | 187� (12–210) | 1�.1 | 483� (45–680) | 0�.9 |
| Complex II–III | 467�.3(179�.2–546�.0) | 1�.2 | 418� (189–433) | 1�.3 | 1082� (680–1468) | 0�.4 |
| Complex IV | 17�.0� (12�.0–32�.0) | −0�.4 | 15� (11–32) | −0�.5 | 39� (36–100) | −1�.0 |
| Citrate synthase | 1117� (497–1473) | 1�.6 | NA | NA | NA | NA |
| LIVER | ||||||
| Complex I | 7�.3� (14�.4–56�.0) | −3�.0 | 76� (162–730) | −3�.1 | 60� (68–252) | −2�.6 |
| Complex II | 122�.2� (174�.7–309�.8) | −2�.4 | 1264� (2304–3311) | −4�.3 | NA | NA |
| Complex III | 26�.4� (13�.8–27�.6) | 0�.9 | 273� (128–315) | 0�.6 | 216� (50–118) | 1�.9 |
| Complex II–III | 146�.7� (10�.8–107�.3) | 1�.5 | 1519� (138–1062) | 1�.5 | 1202� (62–383) | 2�.7 |
| Complex IV | 4�.5� (0�.5–3�.2) | 1�.9 | 47� (6–35) | 1�.8 | 37� (3–13) | 2�.3 |
| Citrate synthase | 96�.5� (59�.5–109�.3) | 0�.6 | NA | NA | NA | NA |
| FIBROBLAST | ||||||
| Complex I | 79�.4� (48�.9–120�.1) | 0�.0 | 133� (151–387) | −1�.3 | 181� (255–539) | −1�.9 |
| Complex II | 438�.2� (144�.4–349�.2) | 2�.1 | 736� (471–919) | 0�.8 | NA | NA |
| Complex III | 9�.0� (7�.92–30�.38) | −1�.0 | 15� (21–79) | −1�.5 | 20� (37–118) | −1�.6 |
| Complex II–III | 188�.3� (102�.1–210�.6) | 0�.9 | 316� (265–571) | −0�.6 | 430� (437–812) | −1�.5 |
| Complex IV | 10�.8� (2�.13–6�.84) | 2�.3 | 18� (6–25) | 1�.9 | 25� (12–37) | 1�.8 |
| Citrate synthase | 595�.5� (245�.4–540�.4) | 1�.6 | NA | NA | NA | NA |
Legend: Activities with range of control values are provided�. The patient value is listed as the Z-score expressed in SD of the log transformed normal value population, with bolded values outside 2 SD�. CS is citrate synthase�.