• Journal of Genetic Medicine
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• 제19권2호
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• pp.100-104
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• 2022

The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12). This is a rare case report of CS with DEE-SWAS in a Korean patient.

• Journal of Korean Neurosurgical Society
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• 제59권6호
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• pp.551-558
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• 2016

Malignant glioma cells invading surrounding normal brain are inoperable and resistant to radio- and chemotherapy, and eventually lead to tumor regrowth. Identification of genes related to motility is important for understanding the molecular biological behavior of invasive gliomas. According to our previous studies, Metallothionein 1E (MT1E) was identified to enhance migration of human malignant glioma cells. The purpose of this study was to confirm that MT1E could modulate glioma invasion in vivo. Firstly we established 2 cell lines; MTS23, overexpressed by MT1E complementary DNA construct and pV12 as control. The expression of matrix metalloproteinases (MMP)-2, -9 and a disintegrin and metalloproteinase 17 were increased in MTS23 compared with pV12. Furthermore it was confirmed that MT1E could modulate MMPs secretion and translocation of NFkB p50 and B-cell lymphoma-3 through small interfering ribonucleic acid knocked U87MG cells. Then MTS23 and pV12 were injected into intracranial region of 5 week old male nude mouse. After 4 weeks, for brain tissues of these two groups, histological analysis, and immunohistochemical stain of MMP-2, 9 and Nestin were performed. As results, the group injected with MTS23 showed irregular margin and tumor cells infiltrating the surrounding normal brain, while that of pV12 (control) had round and clear margin. And regrowth of tumor cells in MTS23 group was observed in another site apart from tumor cell inoculation. MT1E could enhance tumor proliferation and invasion of malignant glioma through regulation of activation and expression of MMPs.

• 한국정밀공학회지
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• 제23권1호
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• pp.209-215
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• 2006

The present study purposed to examine differences between 20s and 40s in visuospatial performance, the number of activated yokels and cerebral lateralization using functional Magnetic Resonance Imaging (fMRI). For this study, eight college students in their twenties (21.5 years on the average) and six adults in their forties (45.7 years on the average) participated in the experiment. Functional brain images were taken from 37 MRI using the single-shot EPI method. Compared to the twenties the forties showed lower visuospatial performance and longer reaction time. In addition, compared to the twenties the forties had a smaller number of activated yokels and less cerebral lateralization. The results of this study show that people's visuospatial performance and number of activated yokels decrease with aging. In addition, they also suggest that cerebral lateralization decreases in order to supplement the lowering of visuospatial performance, which in turn symmetrizes the activation of the left and right hemisphere.

• The Korean Journal of Physiology and Pharmacology
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• 제8권4호
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• pp.187-194
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• 2004

Middle cerebral artery occlusion (MCAO) results in cell death by activation of complex signal pathways for cell death and survival. Hypothermia is a robust neuroprotectant, and its effect has often been attributed to various mechanisms, but it is not yet clear. Upstream from the cell death promoters and executioners are several enzymes that may activate several transcription factors involved in cell death and survival. In this study, we immunohistochemically examined the phosphorylation of mitogen-activated protein kinase, extracellular signal-regulated kinase (ERK), c-Jun N-terminal kinase (JNK) and p38 kinase during early period of the ischemic injury, following 2 hours (h) of transient MCAO. Increased phosphorylation of ERK and p38 was observed in the vessels at 3 h, neuron-like cells at 6 and 12 h and glia-like cells at 12 h. Activation of JNK was not remarkable, and a few cells showed active JNK following ischemia. Phosphorylation of Elk-1, a transcription factor, was reduced by ischemic insult. Hypothermia attenuated the activation of ERK, p38 and JNK, and inhibited reduction of Elk-1. These data suggest that signals via different MAPK family members converge on the cell damage process and hypothermia protects the brain by interfering with these pathways.

• The Korean Journal of Physiology and Pharmacology
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• 제5권5호
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• pp.373-380
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• 2001

The action of opioid on the hyperpolarization-activated cation current $(I_h)$ in substantia gelatinosa neurons were investigated by using whole-cell voltage-clamp recording in rat spinal brain slices. Hyperpolarizing voltage steps revealed slowly activating currents in a subgroup of neurons. The half-maximal activation and the reversal potential of the current were compatible to neuronal $I_h.$ DAMGO $(1\;{\mu}M),$ a selective- opioid agonist, reduced the amplitude of $I_h$ reversibly. This reduction was dose-dependent and was blocked by CTOP $(2\;{\mu}M),$ a selective ${\mu}-opioid$ antagonist. DAMGO shifted the voltage dependence of activation to more hyperpolarized potential. Cesium (1 mM) or ZD 7288 $(100\;{\mu}M)$ blocked $I_h$ and the currents inhibited by cesium, ZD 7288 and DAMGO shared a similar time and voltage dependence. These results suggest that activation of ${\mu}-opioid$ receptor by DAMGO can inhibit $I_h$ in a subgroup of rat substantia gelatinosa neurons.

• 대한의용생체공학회:학술대회논문집
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• 대한의용생체공학회 1998년도 추계학술대회
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• pp.291-292
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• 1998

The purpose of the study was aimed to evaluate the BOLD contrast fMRI in occipital lobe and compare this imaging with metabolite changes based on $^1H$ MRS and MRSI before and after visual stimulation. As a result, the activation map were sucessfully produced by thresholding with minimum cross-correlate value of 0.45. In MRS, NAA/Cr ratio is almost same. however, latate was elevated almost 9 times higher than before activation. Lactate metabolic images were consistent with the BOLD effect map. The BOLD contrast fMRI is not enough to detect the activation area in human brain. so, the other modality was required such as lactate metabolic map.

• Journal of Ginseng Research
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• 제35권1호
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• pp.80-85
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• 2011

Ginseng, a traditional medicine in Asian countries, is known to prevent various neuropathologic diseases such as Alzheimer's. Ginseng total saponins (GTS) in particular are one of the most effective ginseng extract compounds for neuroprotection. However, their protective effects on astrocytes are rarely reported. In pathological circumstances, astroglial activation plays a pivotal role in neuroinflammation. Subsequently, neuroinflammation induced by activated astrocytes causes brain damage. The purpose of the present study was to determine the suppressive effects of GTS on astroglial activation in lipopolysaccharide (LPS)-stimulated rat primary astrocytes. Astrocytes treated for 24 h with LPS demonstrated suppressed glialfibrillary acidic protein expression in a dose-dependent manner in the presence of GTS. GTS reduced production of proinflammatory cytokines such as tumor necrosis factor-${\alpha}$ and interleukin-1${\beta}$ and inhibited the level of inducible nitric oxide synthase, and cyclooxygenase-2 in LPS-stimulated astrocytes. Furthermore, GTS suppressed intracellular reactive oxygen species production. These modulations due to GTS may indicate neuroprotective antiinfl ammatory properties which may in turn be related to improvements in neurological performance.

• 약학회지
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• 제41권6호
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• pp.773-781
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• 1997

The activity of $Ca^{2+}$calmodulin (CaM)-dependent protein kinase Ia (CaM kinase Ia) is shown to be regulated through direct phosphorylation by CaM kinase I kinase (CaMK IK). In the present study, three distinct CaMKIK peaks were separated from Q-Sepharose colunm chromatography of pig brain homogenate using a Waters 650 Protein Purification System. The purified CaMKIK from the major peak potently and rapidly enhanced CaM kinase Ia activity, reaching a maximal stimulation within 2min at the concentrations of 12-15nM. The activated state of CaM kinase Ia is characterized by a markedly enhanced $V_{max}4 as well as significantly decreased $K_m\;and\;K_a$ values toward peptide substrate and CaM, respectively. These observations suggest the activation process of CaM kinase Ia. The phosphorylation of CaM kinase Ia by CaMKIK may induce its conformational change responsible for the alterations in the kinetic properties, which ultimately leads to the rapid enzyme activation.

• 한국정보통신학회:학술대회논문집
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• 한국정보통신학회 2021년도 추계학술대회
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• pp.97-99
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• 2021

최근 고령화 사회가 지속됨에 따라, 치매(Dementia)에 대한 관심이 높아지고 있다. 그 중에서 알츠하이머병(Alzheimer's disease)는 전체 치매 환자의 50~60%로 가장 많은 비율을 차지하는 퇴행성 뇌질환으로, 현재 의료계에선 알츠하이머병에 대한 명확한 예방법 및 치료법에 대해 내놓지 못하고 있으며, 치매 발병 전 조기 치료 및 조기 예방법에 대한 중요성이 강조되고 있다. 본 논문에서는 정상인과 알츠하이머병에 걸린 환자의 MRI 데이터셋을 활용하여 컨볼루션 신경망을 중심으로 여러 가지 활성화 함수를 접목시켜, 가장 효율적인 활성화 함수를 찾고자 한다. 또한 알츠하이머 치매분류 모델링을 통해 향후 의료분야에 적합한 치매 구분 모델링으로 활용하고자 한다.

• 대한약학회:학술대회논문집
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• 대한약학회 2003년도 Proceedings of the Convention of the Pharmaceutical Society of Korea Vol.1
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• pp.198-199
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• 2003

Chronic inflammatory processes are associated with pathology of Alzheimer's disease(AD). The expression of both cyclooxygenase-2(COX-2) and phospholipase A2(PLA2) appears to be strongly activated during AD, indicating the importance of inflammatory gene pathways as a response to brain injury. Stimulation of heterotrimeric G protein-coupled receptors including muscarinic receptors activates cytosolic PLA2 and receptor-mediated activation of PLA2 generates free fatty acids (i.e., arachidonic acid). (omitted)