We performed an immunohistochemical study on the estrogen receptor alpha (ER-alpha) distribution in the cerebellum of a human neonate with multiple congenital anomalies, that had been acquired during autopsy. Although the exact pathology in the brain was not clearly elucidated in this study, an unidentified stressful condition might have induced the astrocytes into reactive states. In this immunohistochemical study on the neonatal cerebellum with multiple congenital anomalies, intense ER-alpha immunoreactivities (IRs) were localized mainly within the white matter even though ER-alpha IRs were known to be mainly localized in neurons. Double immunohistochemical staining showed that ER-alpha IR cells were reactive astrocytes, but not neurons. Interestingly, there were differences in the process length among the reactive astrocytes showing ER-alpha IRs. Our quantitative data confirmed that among the glial fibrillary acidic protein (GFAP)-expressing reactive astrocytes, the cells exhibiting intense ER-alpha IRs have much longer cytoplasmic processes and relatively weaker GFAP IRs. Taken together, the elongated processes of reactive astrocytes might be due to decreased expression of GFAP, which might be induced by elevated expression of ER-alpha even though the elucidation of the exact mechanism needs further studies.
Abnormalities, Multiple/*pathology ; Astrocytes/*metabolism ; Autopsy ; Brain/pathology ; Cerebellum/*metabolism ; Cytoplasm/metabolism ; Estrogen Receptor alpha/*metabolism ; Female ; Gastrointestinal Diseases/congenital/*pathology ; *Gene Expression Regulation ; Glial Fibrillary Acidic Protein/metabolism ; Humans ; Immunohistochemistry/methods ; Infant, Newborn ; Urogenital Abnormalities/*pathology

BACKGROUNDS: Free radical theory showed that aging might be correlating with the accumulation of oxidative damage into biomolecules of animals. Since the process was also observed in some neurodegenerative disease, the study on the subject could improve our understanding of the aging process. In this regard, our study laid focus on the effect of various anti-oxidants on the cells including mutant Cu/Zn-superoxide dismutase, which was reported to be deficient in various diseases or aging processes. METHODS: We tested retinol, Vitamin C, E and coenzyme Q10 on mutant Cu/Zn-superoxide dismutase(A4V) motor neuron cells using immunocytochemical study and accompanying statistical analysis. RESULTS: During our trial on the hydrogen peroxide treatment on A4V mutant cells, Vitamin A and C did not show any defensive effect on oxidative stress induced A4V cells while Vitamin E and coenzyme Q10 exhibited meaningful inhibitory effect against apoptosis relating protein expression. CONCLUSION: In case of oxidative damages induced by Cu/Zn-superoxide dismutase deficiencies, the protective role of some anti-oxidants like Vitamin E or coenzyme Q10 should be positively considered in forthcoming studies.
Aging ; Amyotrophic Lateral Sclerosis ; Animals ; Apoptosis* ; Ascorbic Acid ; Hydrogen Peroxide ; Immunohistochemistry ; Motor Neurons* ; Neurodegenerative Diseases ; Oxidative Stress ; Vitamin A ; Vitamin E ; Vitamins