ChronologicaI Observations of HistologicaI Changes, Cytochrome Oxidase Activity and Copper Level in the Brain of the Postnatal Brindled Mouse
| Autor: | Saburo Usutani, Kazuyuki Kida, Mamoru Asada, Noriaki Yoshimura, Masahiko Nishimura |
|---|---|
| Rok vydání: | 1990 |
| Předmět: |
Male
medicine.medical_specialty Cerebellum Time Factors chemistry.chemical_element Degeneration (medical) Pathology and Forensic Medicine Electron Transport Complex IV Mice Mice Neurologic Mutants Internal medicine medicine Animals chemistry.chemical_classification Mice Inbred C3H Kidney Cerebrum Brain General Medicine Copper Microscopy Electron Crista Enzyme Endocrinology medicine.anatomical_structure chemistry Brainstem |
| Zdroj: | Pathology International. 40:383-390 |
| ISSN: | 1440-1827 1320-5463 |
| DOI: | 10.1111/j.1440-1827.1990.tb01577.x |
| Popis: | Neuropathological and enzyme-histochemical studies were performed on brindled mouse hemizygotes (BMs) and normal littermates at the age of 2 days, 7 days, 11 days and 14 days, together with an investigation of their tissue cop per levels. A greatly increased copper concentration was confirmed in the kidney and intestine and a greatly reduced concentration in the liver and brain of BMs. The copper concentration in the brain increased gradually with age in the normal littermates, whereas this did not occur in BMs. There was no significant difference in the tissue copper concentration between the cerebrum and the cerebellum brainstem in BMs or in normal littermates. Light and electron microscopy of the BM brain revealed progressive neuronal degeneration in association with increased mito-chondrial changes (ballooning and crista disintegration). Enzyme histochemical examinations demonstrated a progressive comparative decrease (i.e., an increased difference from normal) of cytochrome oxidase activity in the BM brain. These data suggest that progressive degeneration of the brain in Menkes’disease is attributable to mitochon-drial degeneration caused by a comparative decrease of both copper concentration and cytochrome oxidase activity in the brain. |
| Databáze: | OpenAIRE |
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