| Project/Area Number |
60570448
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Jichi Medical School. |
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Principal Investigator |
KODAMA Hiroko (1986) Department of Pediatrics, Jichi Medical School, 医学部, 講師 (00093386)
鴨下 重彦 (1985) 自治医科大学, 医学部, 教授
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| Co-Investigator(Kenkyū-buntansha) |
HAMANO Yuji Department of Pediatrics, Jichi Medical School, 医学部, 助手
OKABE Ichiro Department of Pediatrics, Jichi Medical School, 医学部, 助手 (80177071)
MIYAO Masutomo Department of Pediatrics, Jichi Medical School, 医学部, 講師 (90137711)
KAMOSHITA Shigehiko Department of Pediatrics, Tokyo University School of Medicine, 医学部, 教授 (60048973)
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| Project Period (FY) |
1985 – 1986
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| Project Status |
Completed (Fiscal Year 1986)
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| Budget Amount *help |
¥1,600,000 (Direct Cost: ¥1,600,000)
Fiscal Year 1986: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 1985: ¥1,000,000 (Direct Cost: ¥1,000,000)
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| Keywords | Reye's syndrome / ornithine transcarbamylase / immunocytochemistry / プロティンA・ゴールド法 |
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| Research Abstract |
The etiology and pathogenesis of Reye's syndrome remains obsure. The characteristic findings are mitochondrial damage, decrease of the activity of mitochondrial enzymes and fatty accumulation in the viscera. Woodfin et al. reported that mitochondrial enzymes are released from the mitochondria to the cytosol in the liver of Reye's syndrome. However the localization of the mitochondrial enzyme in Reye's syndrome has not been investigated. In the present study. we investigated the localization of ornithine transcarbamylase (OTC). as a marker of mitochondrial enzymes, in the liver of the patients with Reye's syndrome or Reye like syndrome immunochemically using light and electron microscope. In the patients with Reye like syndrome, some were diagnosed as OTC deficiencey. For the light microscopy, small tissue slices of the liver were embedded in Epon and stained by anti-bovine OTC rabbit IgG and the immunoenzyme technique. For the electron microscopy, slices of liver tissues were embedded in Lowicryl K4M and labeled by anti-bovine OTC rabbit IgG and the protein A-gold technique. The liver cells of the male patients with OTC deficiency were not stained for OTC, while those of the female patients with OTC deficiency showed a cellular mosaic consisting of OTC-positive and OTC-negative cells. These results indicate that immunocytochemical examination of liver OTC is useful for diagnosis of OTC deficiency. In the electron microscopic observation of the liver cells of Reye's syndrome, gold particles representing the anti-genic sites for OTC were not detected in the cytosol, but confined in the mitochondrial matrix. Those results show that liver mitochondrial enzymes are not released from mitochondria to the cytosol, but confine in the mitochondria in Reye's syndrome.
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