Hepatic Failure and Enhanced Oxidative Stress in Mitochondrial Diabetes

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Author(s)

    • TAKAHASHI YUTAKA
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • IIDA KEIJI
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • TAKENO RYOKO
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • KITAZAWA RIKO
    • Division of Pathology, Department of Pathology&Microbiology, Kobe University Graduate School of Medicine
    • KITAZAWA SOHEI
    • Division of Pathology, Department of Pathology&Microbiology, Kobe University Graduate School of Medicine
    • KITAMURA HIDETSUNA
    • Division of Cardiovascular and Respiratory Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • FUJIOKA YOSHIO
    • Division of Cardiovascular and Respiratory Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • YAMADA HIROYUKI
    • Division of Diabetes, Digestive, and Kidney Diseases, Department of Clinical Molecular Medicine, Kobe University Graduate School of Medicine
    • KANDA FUMIO
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • OHTA SHIGEO
    • Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Graduate School of Medicine, Nippon Medical School
    • NISHIMAKI KIYOMI
    • Department of Biochemistry and Cell Biology, Institute of Development and Aging Sciences, Graduate School of Medicine, Nippon Medical School
    • FUJIMOTO MASAYO
    • Division of Pathology, Department of Pathology&Microbiology, Kobe University Graduate School of Medicine
    • KONDO TAKESHI
    • Division of Pathology, Department of Pathology&Microbiology, Kobe University Graduate School of Medicine
    • IGUCHI GENZO
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • TAKAHASHI KENTARO
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine
    • OKIMURA YASUHIKO
    • Department of Basic Allied Medicine, Kobe University School of Medicine
    • CHIHARA KAZUO
    • Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine

Abstract

Mitochondrial diabetes is characterized by diabetes and hearing loss in maternal transmission with a heteroplasmic A3243G mutation in the mitochondrial gene. In patients with the mutation, it has been reported that hepatic involvement is rarely observed. We demonstrated a case of hypertrophic cardiomyopathy and hepatic failure with mitochondrial diabetes. To clarify the pathogenesis we analyzed the mitochondrial ultrastructure in the myocytes, the reactive oxygen species (ROS) production in the liver and the status of heteroplasmy of the mitochondrial A3243G mutation in the organs involved. In cardiomyocytes and skeletal muscle, electron microscopic analysis demonstrated typical morphological mitochondrial abnormalities. Immunohistochemical analysis demonstrated enhanced ROS production associated with marked steatosis in the liver, which is often associated with mitochondrial dysfunction. Analysis of the A3243G mutation revealed a substantial ratio of heteroplasmy in these organs including the liver. The presence of steatosis and enhanced oxidative stress in the liver suggested that hepatic failure was associated with mitochondrial dysfunction.<br>

Journal

  • Endocrine Journal

    Endocrine Journal 55(3), 509-514, 2008-06-01

    The Japan Endocrine Society

References:  12

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