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Table 1 Respiratory chain analysis of patient's skeletal muscle tissue

From: Neonatal presentation of ventricular tachycardia and a Reye-like syndrome episode associated with disturbed mitochondrial energy metabolism

Enzyme

Complex

Activity

(μmol/min-1/g-1)

Normal reference mean ± 1 SD

Cytochrome c oxidase

IV

2.56*

(2.8 ± 0.52)

Succinate cytochrome c reductase

II + III

0.55

(0.7 ± 0.23)

NADH cytochrome c reductase

I + III

1.44

(1.02 ± 0.38)

NADH dehydrogenase

I

29.32

(35.48 ± 7.07)

Citrate synthase

 

15.01

(9.88 ± 2.55)

Succinate dehydrogenase

II

1.15

(1 ± 0.53)

  1. NADH = reduced nicotinamide adenine dinucleotide * Data represent the mean of 2 independent analyses on different muscle homogenates
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BMC Pediatrics

ISSN: 1471-2431

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