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. 2022 Sep 7;43(12):1970–1978. doi: 10.1002/humu.24453

Table 1.

Spectrophotometric analysis of oxidative phosphorylation (OXPHOS) enzyme activities in muscle and liver from X1

Muscle Liver
Enzyme

Residual Activity

(%)

CS ratio

(%)

CII ratio

(%)

Residual Activity

(%)

CS ratio

(%)

CII ratio

(%)

I

221

(45−173)

182

(70−149)

141

(66−147)

768

(82−118)

218

(77−124)

327

(92−121)

II

160

(58−138)

127

(80−109)

236

(88−119)

67

(80−126)

III

106

(44−175)

81

(30−171)

65

(31‐161)

74

(68‐135)

21

(61−136)

31

(76–125)

IV

97

(51−139)

78

(77−116)

61

(83‐126)

70

(64−123)

20

(68−130)

29

(65–120)

CS

125

(66−138)

346

(94−110)

Note: Activities of OXPHOS enzyme complexes I, II, III, IV and citrate synthase (CS) are expressed as % relative to protein (residual activity), citrate synthase (CS ratio) and CII (CII ratio) of control samples.