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

Abstract:

: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 micrograms 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 cortex, 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.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Yamano T,Shimada M,Onaga A,Kawasaki H,Iwane S,Ono K,Nishimura M

doi

10.1007/BF00689595

subject

Has Abstract

pub_date

1988-01-01 00:00:00

pages

574-80

issue

6

eissn

0001-6322

issn

1432-0533

journal_volume

76

pub_type

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