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This report from France illustrates the importance
and value of coenzyme Q10 (ubiquinone), especially concerning respiratory
function.
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The authors note that the respiratory-chain
deficiencies are a broad group of largely untreatable diseases.
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There is a subclass of patients with these disorders
who suffer from a coenzyme Q10 deficiency.
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Deficiency of coenzyme Q10-dependent respiratory-chain
activities was identified in muscle biopsy, circulating lymphocytes,
and cultured skin fibroblasts of 2 siblings with this disorder.
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Undetectable coenzyme Q10 in cultured fibroblasts
supported the diagnosis of widespread coenzyme Q10 deficiency.
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The patients were treated with oral CoQ10 (5
mg/kg daily), which resulted in a substantial improvement of their
condition over 3 years of therapy.
The authors conclude that particular attention should
be paid to the fact that some patients with respiratory-chain enzyme deficiency
may be successfully treated by oral CoQ10.
Lancet
August 18, 2000; 356: 391-95
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