Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes

Table 2 Biochemical analysis of patient fibroblasts and muscle biopsies

OXPHOS activities (spectrophotometry)	I	II	III	IV	V	G3P + III	II + III	CS	CoQ10 quantity (LC-MSMS)	CoQ10
Fibroblast measurements
Control values (nmole/min/mg of proteins)	9.0–27.1	21.0–54.0	62.0–176.2	109.9–350.0	22.0–46.2	6.5–23.0	15.0–37.2	74.7–161.1	Control values (pmole/mg of proteins)	43.0–120.8
P01	11.2	27.7	89.7	29.2	33.5	2.3	7.5	156.2	P01	1.4
P03	11.5	18.5	21.3	177.7	34.1	4.1	12.5	106.7	P03	65.4
P04	7.5	18.6	40.2	108.2	30.5	4.7	8.6	95.0	P04	9.7
P06	11.6	20.6	47.9	78.1	37.6	5.5	10.8	116.6	P06	5.9
P09	12.0	13.4	53.4	57.0	15.8	4.1	8.9	80.9	P09	62.0
P10	13.5	22.9	54.4	65.0	28.3	5.4	12.0	148.2	P10	55.9
P12	10.9	17.6	76.6	173.3	25.0	4.4	10.1	102.5	P12	62.1
P16	11.2	20.7	57.4	134.9	38.3	6.1	14.5	124.0	P16	5.7
P17	12.9	20.0	61.5	181.7	29.3	5.6	14.7	130.3	P17	58.1
P18	14.4	22.5	39.4	78.9	39.3	5.5	13.2	147.0	P18	58.2
Muscle biopsy measurements
Control values (nmole/min/mg of proteins)	11.0–32.0	22.0–65.0	109.0–236.0	93.0-347.0	40.0–89.0	14.0–50.0	20.0–50.0	82.0–234.0	Control values (pmole/mg of proteins)	17.8 –22.2
P02	6.7	21.5	130.4	56.9	32.5	7.4	10.8	113.4	P02	16.0
P05	5.7	21.2	28.9	59.4	12.7	10.9	15.2	122.2	P05	35.5
P07	4.2	28.6	108.8	170.4	50.0	10.5	16.7	272.4	P07	25.9
P08	10.9	14.1	102.5	92.8	63.3	10.8	17.0	116.5	P08	5.7
P11	25.7	29.7	157.6	80.2	45.0	13.6	19.6	109.9	P11	6.2
P13	7.6	28.9	112.7	212.7	58.4	9.4	13.4	192.6	P13	22.4
P14	15.5	26.6	31.6	154.5	32.8	13.7	13.2	100.5	P14	22.2
P15	16.2	20.0	92.3	191.7	39.8	11.9	17.5	86.1	P15	7.1

Respiratory chain enzyme activities were measured spectrophotometrically. Results are expressed as absolute values for controls or patients (in nanomoles of substrate per minute per milligram of protein). CoQ₁₀ quantity was measured by LC-MSMS. Results are expressed as absolute values for controls or patients (in picomoles per milligram of protein). Abnormal values are shown in italics
OXPHOS oxidative phosphorylation; LC-MSMS liquid chromatography coupled with tandem mass spectrometry detection

ISSN: 0717-6287