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Article

Complex I Defect in muscle from patients with Huntington's disease

1 Mar 1998

Abstract excerpt

We found a variable defect of complex I of the mitochondrial respiratory chain, ranging in severity from 25% to 63% of control values, in muscle of patients with Huntington's disease (HD). The most severe defect was observed in the patient with the greatest expansion of CAG triplets. Muscle morphology showed myopathic changes such as moth-eaten fibers, angulated fibers, increased subsarcolemmal oxidative...

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PMID: 9506560
DOI: 10.1002/ana.410430321

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