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Table 3 Specific enzyme analysis of ADOA patient and control mitochondria

From: Mitochondrial oxidative phosphorylation in autosomal dominant optic atrophy

 

Mean specific enzyme activities

nmol/min/mg mitochondrial protein

 

Complex I

Complex III

Complex IV

CS a

aOPA1+ (n = 6)

    

P2

82

1076

884

1410

P3

274

976

568

2074

P4

294

928

1005

1693

P6

86

1313

970

918

P7

52

1078

974

846

P8

122

845

828

1018

Mean ± SD

152 ± 105

1036 ± 162

872 ± 162

1327 ± 488

aOPA1- (n = 10)

    

Mean ± SD

190 ± 144

945 ± 174

810 ± 204

1154 ± 650

Control (n = 10)

    

Mean ± SD

170 ± 64

886 ± 179

893 ± 271

1304 ± 364

  1. aAbbreviations used above are CS, citrate synthase, OPA1+, OPA1-positive patients, and OPA1-, OPA1-negative patients