Acta Neuropathologica

, Volume 76, Issue 6, pp 574–580 | Cite as

Electron microscopic study on brain of macular mutant mouse after copper therapy

  • T. Yamano
  • M. Shimada
  • A. Onaga
  • H. Kawasaki
  • S. Iwane
  • K. Ono
  • M. Nishimura
Regular Papers


The hemizygote of the macular mutant mice, which is clinically and neuropathologically considered to be a model of Menkes kinky hair disease (MKHD), were injected intraperitoneally four times with 10, 20, 20 and 30 μg of cupric chloride on days 4, 6, 8 and 10 after birth, respectively. Their cerebral and cerebellar cortices were chronologically examined by electron microscopy. In the cerebral cortes, only a few abnormal mitochondria with electron-lucent matrix and short peripherally located cristae were scattered in the neurons on day 14, and these had almost entirely vanished after day 21. In the cerebellar cortex, abnormal mitochondria were frequently found on day 14 in the dendrites of the Purkinje cells, whereas they were only occasionally observed in their cytoplasm. Those in the dendrites had decreased in number on day 30, and only a few of them were seen in the cerebellum after day 45. These results show that the copper therapy reduced ultrastructural abnormalities in the hemizygote of this mutant mouse.

Key words

Macular mouse Menkes kinky hair disease Copper therapy Mitochondrial abnormalities 


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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • T. Yamano
    • 1
  • M. Shimada
    • 1
  • A. Onaga
    • 1
  • H. Kawasaki
    • 1
  • S. Iwane
    • 1
  • K. Ono
    • 1
  • M. Nishimura
    • 2
  1. 1.Department of PediatricsShiga University of Medical ScienceOtsu-shi, Shiga-kenJapan
  2. 2.Animal Laboratory CenterHamamatsu Medical CollegeShizuokaJapan

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