Deficiency of subunits of complex I and mitochondrial encephalomyopathy | Zendy

Ichiki Takashi | Zendy; Tanaka Masashi | Zendy; Nishikimi Morimitsu | Zendy; Suzuki Hiroshi | Zendy; Ozawa Takayuki | Zendy; Kobayashi Masanori | Zendy; Wada Yoshiro | Zendy

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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.

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