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Publication : Histochemical study of mitochondrial enzymes in cerebellar cortex of macular mutant mouse, a model of Menkes kinky hair disease.

First Author  Kumode M Year  1994
Journal  Acta Neuropathol Volume  87
Issue  3 Pages  313-6
PubMed ID  8009964 Mgi Jnum  J:19578
Mgi Id  MGI:67697 Doi  10.1007/BF00296748
Citation  Kumode M, et al. (1994) Histochemical study of mitochondrial enzymes in cerebellar cortex of macular mutant mouse, a model of Menkes kinky hair disease. Acta Neuropathol (Berl) 87(3):313-6
abstractText  The cerebellar Purkinje cells in the hemizygote of the macular mutant mouse contain numerous abnormal mitochondria which show a marked decrease in cytochrome c oxidase activity. Using histochemical methods we studied the activity of other mitochondrial enzymes, such as NADH diaphorase and succinic dehydrogenase, in the cerebellar cortex of this mutant mouse. Such activities were markedly increased in the Purkinje cells, especially in the soma and stem dendrite, from 10 days after birth in the hemizygote as compared with findings in normal littermates. These results were considered to be due to an increased number of abnormal mitochondria.
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