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Copper Contents and Pathological Changes in Various Organs of Macular Mouse
Author(s) -
KATSURA Tadahiko,
KAWASAKI Hisaki,
YAMANO Tsunekazu,
SHIMADA Morimi
Publication year - 1988
Publication title -
congenital anomalies
Language(s) - English
Resource type - Journals
eISSN - 1741-4520
pISSN - 0914-3505
DOI - 10.1111/j.1741-4520.1988.tb00581.x
Subject(s) - emaciation , ataxia , copper deficiency , pathological , dry weight , kidney , wet weight , chemistry , endocrinology , copper , medicine , biology , pathology , anatomy , zoology , botany , organic chemistry , neuroscience
The hemizygote of the macular mutant mouse ( Ml/y ) is considered to be a model of Menkes kinky hair disease (MKHD). It suffers from seizure, ataxia and emaciation, and dies around day 15 after birth. However hemizygotes which were injected with 10, 20, 20 and 30 μg of cupric chloride on days 4, 6, 8 and 10, respectively were freed from these clinical manifestations. Copper contents in the cerebrum, cerebellum, brain stem and liver from untreated Ml/y were significantly lower than those from normal littermate (+/ y ); 1.9±0.4, 3.8±1.0, 2.5±0.6 and 9.3±2.5 /μg/g dry weight in Ml/y , and 7.0±0.7, 11.3±3.3, 8.6±1.7 and 29.1±10.4 μg/g dry weight in +/y , respectively, on day 14. On the contrary, copper contents in the kidney and intestine from Ml/y were higher than those from Ml/y 35.5±10.9 and 24.3±7.7 μg/g dry weight from Ml/y , and 9.4±1.2 and 6.1±1.3 Mg/g dry weight from +/y , respectively, on day 14. By electron microscopic observation, although abnormal mitochondria were now and again observed in the neurons of the spinal cord, pathological changes were not seen in the liver, kidney and intestine from Ml/y. In the treated Ml/y , the copper contents of the brain and liver were raised to between those of the Ml/y and of the +/y. The copper contents in the kidney and intestine had increased much more in the treated Ml/y , and were 5.3 and 1.6 times more than those in the Ml/y , respectively, on day 14. These results show that the distribution of copper in various organs of Ml/y is quite similar to that of the patient with MKHD, and that the copper injection raised the level of copper content in the nervous system, thus improving the clinical features of Ml/y.