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

Summary

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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References

  1. 1.
    Aikawa H, Suzuki K, Iwasaki Y (1983) Ultrastructural observation on the thalamic neuronal inclusions in young mice. Acta Neuropathol (Berl) 59:313–318Google Scholar
  2. 2.
    Caviness VS (1975) Architectonic map of neocortex of the normal mouse. J Comp Neurol 164:247–264Google Scholar
  3. 3.
    Fraser H, Smith W, Gray EW (1970) Ultrastructural morphology of cytoplasmic inclusions within neurons of aging mice. J Neurol Sci 11:123–127Google Scholar
  4. 4.
    Hansson H-A (1981) Purkinje nerve cell changes caused by electric fields—Ultrastructural studies on long-term effects on rabbits. Med Biol 59:103–110Google Scholar
  5. 5.
    Herdon RM (1964) Lamellar bodies, an unusual arrangement of the granular endoplasmic reticulum. J Cell Biol 20:338–342Google Scholar
  6. 6.
    Hirano A, Llena JF, Ghatak NR (1977) Fine structure of the cerebellar cortex in Menkes kinky hair disease. X-chromosome-linked copper malabsorption. Arch Neurol 34:52–56Google Scholar
  7. 7.
    Kawasaki H, Onaga A, Yamano T, Shimada M, Nishimura M (1987) Golgi study on brain of macular mutant mouse as a model of Menkes kinky hair disease. Acta Neuropathol (Berl) 72:349–354Google Scholar
  8. 8.
    Kawasaki H, Yamano T, Iwane S, Shimada M (1988) Golgi study on macular mutant mouse after copper therapy. Acta Neuropathol (in press)Google Scholar
  9. 9.
    Landis DMD, Rogenberg RN, Landis SC, Schut L, Nyhan WL (1974) Olivopont-cerebellar degeneration. Clinical ultrastructural abnormalities. Arch Neurol 31:295–307Google Scholar
  10. 10.
    Menkes JH, Alter M, Steigleder G, Weakley DR, Sung JH (1962) A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration. Pediatrics 29:764–779Google Scholar
  11. 11.
    Nishimura M (1975) A new mutant mouse, Macular (M1) (in Japanese). Exp Anim (Tokyo) 24:185Google Scholar
  12. 12.
    Onaga A, Kawasaki H, Yamano T, Shimada M, Nishimura M (1987) Light and electron microscopic study on cerebellar cortex of macular mutant mouse as a model of Menkes kinky hair disease. Brain Dev 9:265–269Google Scholar
  13. 13.
    Pena CE (1980) Intracytoplasmic neuronal inclusions in the human thalamus. Light-microscopic, histochemical and ultrastructural observations. Acta Neuropathol (Berl) 52:157–159Google Scholar
  14. 14.
    Wen GY, Wisniewski HM, Shek JW, Loo YH, Fulton TR (1980) Neuropathology of phenylacetate poisoning in rats: an experimental model of phenylketonuria. Ann Neurol 7:557–566Google Scholar
  15. 15.
    Yamano T, Suzuki K (1986) Cerebellar changes of the female mice heterozygous for blindled gene. Acta Neuropathol (Berl) 69:220–226Google Scholar
  16. 16.
    Yamano T, Shimada M, Nakao K, Nakamura T, Wakaizumu S, Kusunoki T (1978) Maturation of Purkinje cells in mouse cerebellum after neonatal administration of cytosine arabinoside. Acta Neuropathol (Berl) 44:41–45Google Scholar
  17. 17.
    Yamano T, Paldino AM, Suzuki K (1985) Ultrastructural and morphometric studies of Purkinje cells of brindled mouse after administration of cupric chloride. J Neuropathol Exp Neurol 44:97–107Google Scholar
  18. 18.
    Yamano T, Shimada M, Kawasaki H, Onaga A, Nishimura M (1987) Clinico-pathological study on macular mutant mouse. Acta Neuropathol (Berl) 72:256–260Google Scholar

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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