• Journal of Life Science
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• v.20 no.9
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• pp.1332-1338
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• 2010

To elucidate the mechanism underlying the regulation of hST8Sia I gene expression in FenR-induced SH-SY5Y cells, we characterized the promoter region of the hST8Sia I gene. Functional analysis of the 5'-flanking region of the hST8Sia I gene showed that the -1146 to -646 region functions as the FenR-inducible promoter of hST8Sia I in SH-SY5Y cells. Site-directed mutagenesis indicated that the NF-&B binding site at -731 to -722 was crucial for the FenR-induced expression of hST8Sia I in SH-SY5Y cells. To investigate which signal transduction pathway was involved in FenR-stimulated induction of hST8Sia I in SH-SY5Y cells, we performed Western blot analysis using phospho-specific antibodies in order to measure their degree of regulatory phosphorylation. Phosphorylations of AKT and RelA (p65) subunit of NF-${\kappa}B$ were significantly elevated in cytosolic and nuclear fractions of FenR-stimulated SH-SY5Y cells, respectively, than in control or DMSO-treated SH-SY5Y cells. These results suggest that FenR induce transcriptional up-regulation of hST8Sia I gene expression through translocation of RelA (p65) subunit of NF-${\kappa}B$ to nucleus by AKT signal pathway in SH-SY5Y cells.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.10 no.3
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• pp.648-653
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• 2009

p62 is a key component of protein aggregates found in brains of neurodegenerative diseases in which oxidative stress is involved in the pathogenesis. p62 was induced in SH-SY5Y, a neuroblastoma cell line, by hydroxydoparnine or $C_2-ceramide$ known to be related to neurodegenerative diseases. The over-expression of p62 showed the neuroprotective effect against the ceramide induced cell death. In addition, p62 became insoluble and cleaved forms as time proceeded after the ceramide treatment, suggesting the mechanism by which p62 is associated with aggregates in neurodegenerative diseases.

• Journal of Korean Neurosurgical Society
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• v.29 no.6
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• pp.836-840
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• 2000

Intramedullary spinal hemagioblastomas usually develope in cervical or thoracic region of spinal cord, but rarely in conus medullaris. We report a case of hemangioblastoma developed in conus medullaris. The 19-year-old male patient presented with slowly progressing low back pain and paresthesia of both legs. MRI and spinal angiography revealed a well-vascularized mass lesion in the conus medullaris with syrinx formation. Total excision of hemangioblastoma was achieved via posterior approach. Postoperatively, patient's walking difficulty was worsened transiently, but it was improved at discharge.

• Journal of Korean Neurosurgical Society
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• v.30 no.2
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• pp.217-220
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• 2001

A rare case of multiple supratentorial and infratentorial hemangioblastomas in a 50-year old man is presented. There were neither manifestations of visceral tumors nor familial history. The two tumors were totally removed in two sessions and the diagnosis in both tumors were hemangioblastomas.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.58-63
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• 1999

Neuroblastoma is a solid tumor derived from neural crest cells of the sympathetic nervous system. It is the most common extracranial solid tumor in children. Although it has the highest rate of spontaneous regression, it has a bad prognosis. Recent reports indicate a much improved outcomes utilizing the multitreatment approaches and early diagnosis as a result of patient screening. We have studied 42 patients managed in the last decade at the Severance and Yongdong Severance Hospitals. The patients were followed until January 1998 and analyzed in terms of age, sex, admission period, stage, diagnostic studies, clinical symptoms, physical findings, operative time, treatment modalities, and survival rate. Twenty eight patients underwent operative procedures, 16 patients had postoperative chemotherapy, and 19 patients had preoperative chemotherapy. Sexual difference was 1.33:1 in favor of males, and 43 % of patients were under one year of age. The patients were initially diagnosed as a result of symptoms and signs. An abdominal mass was the most common clinical finding. Eighty six percent of the patients were in advanced stages (>Stage III of INSS). The 2 year survival rate was 59.2 % and the 5 year survival rate was 29.6 %.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.68-72
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• 2009

Neuroblastoma is the most common extracranial solid tumor in children, and accompanies various clinical symptoms including hypertension. Hypertension is associated with catecholamines secreted from the tumor, and is usually not severe. We report one case of malignant hypertension with cardiac failure in a patient with adrenal neuroblastoma, successfully treated with adrenalectomy. A 3 year-old boy complained of protrusion of the chest wall. Physical examination revealed severe hypertension with cardiac failure. The levels of metabolites of catecholamine were increased in blood (norepinephrine >2000 pg/mL) and urine (norepinephrine 1350.5 ug/day). Abdominal CT showed a 7 cm-sized solid mass arising from the right adrenal gland. After stabilizing the hemodynamics with oral phenoxybenzamine, right adrenalectomy was performed. Pathological diagnosis was a ganglioneuroblastoma. The hypertension and cardiac failure were resolved after tumor removal.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.792-796
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• 2008

Bone pain in a child could be associated with cancer as an initial manifestation of the disease. The childhood malignancies that frequently present bone pain are leukemia, neuroblastoma, and primary bone tumors such as osteosarcoma and Ewing sarcoma. Persistent bone or joint pain associated with swelling, mass, or limitation of motion implies underlying serious causes. Systemic manifestations such as lymphadenopathy, hepatosplenomegaly, fever, fatigue, night sweat, and laboratory abnormalities are also suggestive of malignancy. The index of suspicion tends to be low since less than 1% of children who complain of bone pain are diagnosed as cancer. Nonetheless, pediatricians should be alert to the possibilities of cancer since early detection and prompt treatment might reduce mortality.

• Journal of Oriental Neuropsychiatry
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• v.18 no.1
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• pp.95-110
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• 2007

Objective : The purpose of this study was to investigate molecular basis of neuroprotective effect in total ginsenosides. After H202 induced neurotoxicity, gene expression profiling of SH-SY5Y neuroblastoma cells treated by total ginsenosides is analyzed. Method : After SH-SY5Y cells were cultured, they were damaged by H202 induced oxidative stress. After twenty four hours, experimental group is treated by total ginsenosides and control group is treated by 0.9% saline. A high density cDNA microarray chip is used to analyze the gene expression profiling of SH-SY5Y cells. The Significance Analysis of Microarray method is used for identifying genes on a microarray. Results : 1. According to the results of microarray experiment, 17 genes were up-regulated, 38 genes were down-regulated. 2. Expression of OPHNl, KTANl, ATM, PRKCE, MAPKs genes associated with cell proliferation, neural growth, and the prevention of apoptosis were increased. 3. Change of EPX gene was the greatest among all genes. EPX gene associated with oxidative stress, and tumor suppressor gene ADAM11 were decreased. Conclusion : According to this study, molecular basis of neuroprotective effect of total ginsenosides is as followings: the increase of gene expression associated with cell proliferation, neuron growth, the prevention of apoptotsis and decrease of gene expression associated with oxidative stress and tumor suppressor.

• Korean Journal of Biological Psychiatry
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• v.2 no.2
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• pp.252-256
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• 1995

Corticotropin-releasing factor(CRF)를 분비하는 배양세포주는 CRF의 세포내 조절을 연구하는데 있어 훌륭한 체계가 된다. BE(2)-M17 및 BE(2)-C 세포주는 CRF를 생산하고 분비하며 forskolin 처치에 반응하는 것으로 알려져 있다. 저자들은 이들 세포주에 phorbol 에스테르, 즉 phorbol 12-myristate 13-acetate(TPA) 0.8, 4, 10, 20 nM, 및 nitric oxide(NO)의 신호전달 경로의 자극제(1-${\mu}M$ nitroprusside)와 차단제($1{\mu}M$ nitroprusside+$300{\mu}M$ $N^G$-methyl-D-arginine), 그리고 interleukin-$1{\alpha}$ (IL $1{\alpha}$ ; 4, 20, 100, 500 pM)를 처치하여 CRF를 의미있게 증가시켰으나 세포외 분비는 C 세포에서만 변화되었다. NOS계의 자극제와 차단제는 C 세포의 forskolin 효과를 의미있게 변화시켰다. IL$1{\alpha}$는 두 세포주에 대한 영향이 없었다. 상기 제제들에 대한 이들 세포의 반응이 중추신경계 CRF 신경들과 관련하여 논의되었다.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.56-59
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• 2000

The WDHA syndrome characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and with neurogenic tumors. A 20-month-old girl presenting with symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of the adrenal gland. The serum level of VIP was elevated. After complete excision of the tumor, all symptoms related to the WDHA syndrome were relieved and serum VIP level dropped to normal. The postoperative course was uneventful. The patient was treated with postoperative chemotherapy and radiation therapy. There was no evidence of disease 33 months after operation.