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Energetics and genetics of human mitochondria. Yasuo KAGAWA 1 1Department of Biochemistry, Jichi Medical School pp.567-578
Published Date 1987/8/10
DOI https://doi.org/10.11477/mf.1431905908
  • Abstract
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Human brain is highly vulnerable to both anoxia and hypoglycemia, because neurons depend continuously on mitochondrial metabolism and glycolysis, respectively. Substrates are finally decarboxylated and dehydrogenated by pyruvate dehydrogenase complex and TCA cycle. Human pyruvate dehydrogenase complex was sequenced by DNA analysis in collaboration with Prof. M. Koike. The energy of oxidoreduction is converted into the electrochemical potential difference of protons by electron transport system in mitochondrial inner membrane. Finally, ATP (F0F1) synthase converts the energy of proton flux driven by the potential through F0F1 into the energy of ATP synthesis. In fact, F0F1 incorporated into a planar lipid bilayer produced proton current on ATP hydrolysis (J. Biol. Chem. 261: 9839, 1986). The catalytic center of F1 is the beta subunit. The gene for the human beta subunit was also sequenced in this laboratory and its detailed structure was elucidated. Biogenesis of mitochondria depends on both mitochondrial DNA and nuclear DNA. F0F1, for example, is coded by both nuclear gene (F1 and part of F0) and mitochondrial gene (part of F0). The cytoplasmically synthesized precursor of the beta subunit was shown to be translocated into mitochondria to loose its signal peptide. Polymorphism of mitochondrial DNA of Japanese is very frequent, and that of F1 beta gene was also detected in collaboration with Prof. Tada. Inherited mitochondrial myopathy is discussed in the light of energetics and genetics of mitochondria.


Copyright © 1987, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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