PubMed Health⌕ Search

Biomedical subjects

A Onaga

Publications and source records attributed to A Onaga.

8 recordsLinked to original sources
PubMed1988

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

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.

Animals↗
PubMed1987

Golgi study on brain of macular mutant mouse as a model of Menkes kinky hair disease.

This study was undertaken to elucidate, using the Golgi method, the neuropathological change in the brain of the macular mutant mouse, whose hemizygote (Ml/y) is considered to be a model of Menkes kinky hair disease (MKHD). The hemizygote mice gradually lost weight after 10 days of age and died with emaciation and seizure around day 15. The normal littermate (+/y) was well developed. In the cerebrum, the arborization of pyramidal neurons in the layer V of the Ml/y was the same as that in the +/y on day 10. However, development of arborization in the Ml/y was delayed in comparison with that in the +/y on days 12 and 14. Purkinje cells with several somal sprouts were observed in the cerebellum in both the Ml/y and +/y on day 7. The somal sprouts in the +/y had regressed gradually by day 12, while they were still in the anterior and middle lobes of the Ml/y on day 14. Additionally, the trunks of Ml/y stem dendrites became thicker and a cactus formation was recognized on the branching portion of the dendrites on day 14. Arborization of these abnormal Purkinje cells was distinctly poor compared with that in the +/y. These results suggest that the growth of the neurons is delayed in the Ml/y and simultaneously their cytoskeletal developments are disturbed, especially in the Purkinje cells. There is a close similarity in many respects to the neuropathological change in MKHD.

Animals↗
PubMed1987

Clinico-pathological study on macular mutant mouse.

The macular mutant mouse was clinically and pathologically examined. The hemizygotes began to show white fur color and curly whiskers around postnatal day 3, then seizures and ataxia around day 8, while the normal littermates did not. The hemizygotes also increased weight gradually from birth to day 9, but then showed weight loss and died around day 15 with severe emaciation. These clinical features resembled those in Menkes kinky hair disease. There were no pathological changes in the cerebral cortex in the hemizygotes on day 7. On day 10, two to three clear vacuoles began to appear in a few neurons in the cerebrum. These neurons with vacuoles increased gradually in number and degenerative neurons were also observed by day 14. Ultrastructurally, they corresponded to giant abnormal mitochondria with an electron-lucent matrix and short peripherally located cristae. Other abnormal mitochondria, which were characterized by an electron-dense matrix with tubular or vesicular cristae, were also observed in the cerebral cortical neurons.

Age Factors↗
PubMed1987

Light and electron microscopic study on cerebellar cortex of macular mutant mouse as a model of Menkes kinky hair disease.

The macular mouse is a mutant mouse, the hemizygotes of which show clinical and biochemical abnormalities similar to those in Menkes kinky hair disease (MKHD) in humans. The cerebellar cortex of this mutant suckling mouse was examined by light and electron microscopy. In hemizygotes, the Purkinje cells showed a delay in the maturation of dendrites and somatic spines. Somal sprouts, abnormal mitochondria, and filamentous cytoplasmic inclusions were observed on these cells on day 13. Axonal swellings, containing abnormal mitochondrias were also seen in the inner granular layer. These findings correspond with those of MKHD in humans and those of the brindled mouse, another model mouse of MKHD.

Animals↗
PubMed1987

Electronmicroscopic study on biopsied rectal mucosa in adrenoleukodystrophy.

Rectal mucosal biopsies were performed for seven members of five families with adrenoleukodystrophy (ALD). Many histiocytes contained characteristic cytoplasmic lamellar inclusions that were identical to those seen in adrenocortical cells, brain macrophages, and Schwann cells of affected patients. The ultrastructural features were seen in all patients and also in two asymptomatic younger brothers who had presymptomatic ALD according to assay of very-long-chain fatty acids.

Adrenoleukodystrophy↗
PubMed1986

Quantitative synaptic changes on Purkinje cell dendritic spines of rats born from streptozotocin-induced diabetic mothers.

This study was undertaken to elucidate the quantitative synaptic changes on the dendritic spines of the Purkinje cells of the rats born from streptozotocin-induced diabetic mothers. The brains of the experimental rats including cerebellum decreased in weight and in size on days 1, 30 and 70 as compared to those of the age-matched controls. Ultrastructural observation did not show any significant destructive changes in their cerebellum on days 30 and 70. However, the measurements for the synaptic length and area of dendritic spine of their Purkinje cells on days 30 and 70 showed an increase of about 10% in the former and of about 42% in the latter as compared to those in the age-matched control. These evidences suggest a presence of compensation in the brains thus damaged.

Animals↗