Human mitochondrial complex I dysfunction. |
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Authors: | J M Cooper V M Mann D Krige A H Schapira |
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Institution: | Department of Neuroscience, Royal Free Hospital School of Medicine, London, UK. |
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Abstract: | In humans, complex I dysfunction has been observed in a high percentage of patients with mitochondrial myopathy. Analysis of mitochondria from these patients suggests the function and assembly of complex I is particularly susceptible to abnormalities of mitochondrial DNA, involving either point mutations of tRNA genes or major deletions. The evidence for a complex I defect in Parkinson's disease is accumulating, although the cause of this deficiency or the role it plays in the events that culminate in dopaminergic cell death remains unresolved. |
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