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Deficiency of subunits of complex I and mitochondrial encephalomyopathy
Author(s) -
Ichiki Takashi,
Tanaka Masashi,
Nishikimi Morimitsu,
Suzuki Hiroshi,
Ozawa Takayuki,
Kobayashi Masanori,
Wada Yoshiro
Publication year - 1988
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410230312
Subject(s) - mitochondrial myopathy , mitochondrial encephalomyopathy , lactic acidosis , rotenone , mitochondrion , coenzyme q – cytochrome c reductase , biochemistry , biology , respiratory chain , mitochondrial respiratory chain , melas syndrome , encephalopathy , myopathy , mitochondrial dna , medicine , cytochrome c , genetics , gene
Enzymic activities of the respiratory chain and content of immunochemically detectable subunits in NADH‐ubiquinone oxidoreductase (Complex I) were measured in mitochondria from the skeletal muscles of 4 patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The rotenone‐sensitive NADH‐cytochrome c reductase activity was extremely decreased, ranging from 0% to 27% of the control value. In all patients, the content of subunits of Complex I was also reduced in parallel with the rotenone‐sensitive NADH‐cytochrome c reductase activity. It is suggested that the variation in the degree of deficiency of Complex I subunits could explain the clinical heterogeneity of patients with MELAS.