• BMB Reports
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• 제42권3호
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• pp.136-141
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• 2009

Familial Amyotrophic lateral sclerosis (FALS) is a progressive neurodegenetative disorder induced by mutations of the SOD1 gene. Heat shock protein 27 (HSP27) is well-defined as a stress-inducible protein, however the its role in ALS protection has not yet been established. To investigate the role HSP27 may have in SOD1 mutant-mediated apoptosis, human SOD1 or HSP27 genes were fused with a PEP-1 peptide in a bacterial expression vector to produce a genetic in-frame fusion protein, which was then transduced into cells. We found the purified PEP-1-HSP27 fusion proteins can be transduced efficiently into neuronal cells and protect against cell death by enhancing mutant SOD1 activity. Moreover, transduced PEP-1-HSP27 efficiently prevents protein aggregation produced by oxidative stress. These results suggest that transduced HSP27 fusion protein may be explored as a potential therapeutic agent for FALS patients.

• The Korean Journal of Physiology and Pharmacology
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• 제16권1호
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• pp.43-48
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• 2012

Glutamate excitotoxicity is emerging as a contributor to degeneration of spinal cord motoneurons in amyotrophic lateral sclerosis (ALS). Recently, we have reported that ghrelin protects motoneurons against chronic glutamate excitotoxicity through the activation of extracellular signal-regulated kinase 1/2 and phosphatidylinositol-3-kinase/Akt/glycogen synthase kinase-$3{\beta}$ pathways. Previous studies suggest that activated microglia actively participate in the pathogenesis of ALS motoneuron degeneration. However, it is still unknown whether ghrelin exerts its protective effect on motoneurons via inhibition of microglial activation. In this study, we investigate organotypic spinal cord cultures (OSCCs) exposed to threohydroxyaspartate (THA), as a model of excitotoxic motoneuron degeneration, to determine if ghrelin prevents microglial activation. Exposure of OSCCs to THA for 3 weeks produced typical motoneuron death, and treatment of ghrelin significantly attenuated THA-induced motoneuron loss, as previously reported. Ghrelin prevented THA-induced microglial activation in the spinal cord and the expression of pro-inflammatory cytokines tumor necrosis factor-${\alpha}$ and interleukin-$1{\beta}$. Our data indicate that ghrelin may act as a survival factor for motoneurons by functioning as a microglia-deactivating factor and suggest that ghrelin may have therapeutic potential for the treatment of ALS and other neurodegenerative disorders where inflammatory responses play a critical role.

• 한국정보통신학회:학술대회논문집
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• 한국해양정보통신학회 2006년도 춘계종합학술대회
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• pp.324-327
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• 2006

언어 장애를 가진 사람들 중에서는 언어 장애와 더불어 신체적인 장애를 동반하는 경우도 있다. 특히, 루게릭병이라 불리는 ALS병을 가진 사람들의 경우는 시간이 지날수록 장애의 정도가 변하여, 간병인 혹은 주변의 가족들과의 의사소통이 되었던 사람들도 간단한 의사소통도 어려운 상황이 되는 것이 보편적인 경우이다. 따라서 본 연구개발에서는 루게릭병으로 의사소통이 어려운 분들을 대상으로 의사소통할 수 있는 시스템을 설계 및 구현하였다. 본 시스템은 이러한 사람들의 원활한 의사소통을 위한 것으로, 사용빈도가 높은 단어들을 미리 입력해두고 선택함으로써 의사를 전달하거나, 화상키보드를 통해 확장된 언어를 구현할 수 있도록 한다. 또한 신체의 불편함으로 인해 기존 입력인터페이스를 사용하지 못할 경우를 대비하여 마우스의 클릭이라는 간단한 입력만으로 화면의 내용을 선택할 수 있도록 하였다. 마지막으로 본 논문에서는 이러한 기능들의 구현 및 그 방법에 대해 논해보고, 향후 발전될 방향을 살펴보았다.

• Animal cells and systems
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• 제14권1호
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• pp.1-10
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• 2010

Previously we showed that CHIP, a co-chaperone of Hsp70 and E3 ubiquitin ligase, can promote the degradation of mutant SOD1 linked to familial amyotrophic lateral sclerosis (fALS) via a mechanism not involving SOD1 ubiquitylation. Here we present evidence that CHIP functions in the interaction of mutant SOD1 with 26S proteasomes. Bag-1, a coupling factor between molecular chaperones and the proteasomes, formed a complex with SOD1 in an hsp70-dependent manner but had no direct effect on the degradation of mutant SOD1. Instead, Bag-1 stimulated interaction between CHIP and the proteasome-associated protein VCP (p97), which do not associate normally. Over-expressed CHIP interfered with the association between mutant SOD1 and VCP. Conversely, the binding of CHIP to mutant SOD1 was inhibited by VCP, implying that the chaperone complex and proteolytic machinery are competing for the common substrates. Finally we observed that mutant SOD1 strongly associated with the 19S complex of proteasomes and CHIP over-expression specifically reduced the interaction between S6/S6' ATPase subunits and mutant SOD1. These results suggest that CHIP, together with ubiquitin-binding proteins such as Bag-1 and VCP, promotes the degradation of mutant SOD1 by facilitating its translocation from ATPase subunits of 19S complex to the 20S core particle.

• 한국정보통신학회:학술대회논문집
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• 한국정보통신학회 2018년도 춘계학술대회
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• pp.696-699
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• 2018

본 논문은 FPGA를 이용해서 몸을 움직이지 못하는 루게릭병 환자들을 위해 동공을 추적하여 의사 소통을 할 수 있게 해주는 시스템을 소개한다. OpenCV를 이용한 얼굴 및 눈동자 추적과 DE1-SoC보드를 이용하여 눈의 움직임 검출과 문자를 출력한다. 본 논문에서는 웹캠을 사용하고 눈동자를 추적하고 눈동자의 좌표 값에 따라 눈동자의 움직임을 파악하고 사용자의 의도에 맞는 문자를 출력한다. 비교적 개발비용이 저렴하고 업데이트 가능한 FPGA를 사용하고, 블루투스를 이용하여 핸드폰으로 쉽게 문자를 출력할 수 있는 시스템을 제안한다.

• 한방재활의학과학회지
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• 제26권3호
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• pp.183-189
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• 2016

A 84 year old male patient with amyotrophic lateral sclerosis (ALS) was treated by Jeungsonbaekchulsan and Gakbyeongyeonsu-tang for ten days. In the course of treatments, this patient was evaluated with Roussel Uclaf Casusality Assessment Method (RUCAM), showing results of drug induced liver injury. Herb medicine was immediately discontinued, followed by therapeutic laser treatments once a day for one week combined with 2 weeks of GODEX$^{(R)}$ administration. Transaminase results were lowered and changes in blood test results were time-effective changes. Therapeutic laser therapy could be considered effective on drug induced liver injury with further studies.

• 생명과학회지
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• 제16권5호
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• pp.716-722
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• 2006

Superoxide dismutase (SOD) is physiologically important in regulating cellular homeostasis and apoptotic cell death, and its mutations are the cause of familial amyotrophic lateral sclerosis (FALS). Mitochondrial serine protease HtrA2 has a pro-apoptotic function and has known to be associated with neurodegenerative disorders. To investigate the relationship between genes associated with apoptotic cell death, such as HtrA2 and SOD1, we utilized the pGEX expression system to develop a simple and rapid method for purifying wild-type and ALS-associated mutant SOD1 proteins in a suitable form for biochemical studies. We purified SOD1 and SOD1 (G93A) proteins to approximately 90% purity with relatively high yields (3 mg per liter of culture). Consistent with the result in mammalian cells, SOD1 (G93A) was more insoluble than wild-type SOD1 in E. coli, indicating that research on the aggregate formation of SOD1 may be possible using this pGEX expression system in E. coli. We investigated the HtrA2 serine protease activity on SOD1 to assess the relationship between two proteins. Not only wild-type SOD1 but also ALS-associated mutant SOD1 (G93A) were cleaved by HtrA2, resulting in the production of the 19 kDa and 21 kDa fragments that were specific for anti-SOD1 antibody. Using protein gel electrophoresis and immunoblot assay, we compared the relative molecular masses of thrombin-cleaved GST-SOD1 and HtrA2-cleaved SOD1 fragments and can predict that the HtrA2-cleavage sites within SOD1 are the peptide bonds between leucine 9-lysine 10 (L9-K10) and glutamine 23-lysine 24 (Q23-K24). Our study indicates that SOD1 is one of the substrate for HtrA2, suggesting that both HtrA2 and SOD1 may be important for modulating the HtrA2-SOD1-mediated apopotic cell death that is associated with the pathogenesis of neurodegenerative disorder.

• 약학회지
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• 제55권5호
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• pp.385-390
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• 2011

Bee venom (BV), which is extracted from honeybees, has been used in traditional Korean medical therapy. Glutamate-mediated excitotoxicity contributes to neuronal death in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) or Alzheimer's disease (AD). This study is to investigate the effect of BV on glutamate-induced neurotoxicity on NSC-34 motor neuron cells. To determine the viability of motor neuronal cells, we performed with MTT assays in glutamate-treated NSC-34 cell with BV or without. For the measurement of oxidative stress, DCF assay was used in glutamate-treated NSC-34 motor neuronal cells with BV or without. To investigate the molecular mechanism of BV against glutamate-mediated neurotoxicity in NSC-34 cells, western blot analysis was used. Glutamate significantly decreased cell viability by glutamate dose- or treatment time-dependent manner in NSC-34 cells. However, BV pre-treatment dramatically inhibited glutamate-induced neuronal cell death. Furthermore, we found that BV increased the expression of Bcl-2 protein that is anti-apoptotic protein and reduced the generation of oxidative stress. BV has a neuroprotective role against glutamate neurotoxicity by an increase of anti-apoptotic protein. It suggests that BV may be useful for the reduction of neuronal cell death in neuronal disease models.

• 대한전기학회논문지:시스템및제어부문D
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• 제55권11호
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• pp.502-510
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• 2006

In this paper, we describe implementation of a computer access device for the severly motor-disability. Many people with severe motor disabilities need an augmentative communication technology. Those who are totally paralyzed, or 'locked-in' cannot use conventional augmentative technologies, all of which require some measure of muscle control. The forehead is often the last site to suffer degradation in cases of severe disability and degenerative disease. For example, In ALS(Amyotrophic Lateral Sclerosis) and MD(Muscular dystrophy) the ocular motorneurons and ocular muscles are usually spared permitting at least gross eye movements, but not precise eye pointing. We use brain and body forehead bio-potentials in a novel way to generate multiple signals for computer control inputs. A bio-amplifier within this device separates the forehead signal into three frequency channels. The lowest channel is responsive to bio-potentials resulting from an eye motion, and second channel is the band pass derived between 0.5 and 45Hz, falling within the accepted Electroencephalographic(EEG) range. A digital processing station subdivides this region into eleven components frequency bands using FFT algorithm. The third channel is defined as an Electromyographic(EMG) signal. It responds to contractions of facial muscles and is well suited to discrete on/off switch closures, keyboard commands. These signals are transmitted to a PC that analyzes in a time series and a frequency region and discriminates user's intentions. That software graphically displays user's bio-potential signals in the real time, therefore user can see their own bio-potentials and control their physiological signals little by little after some training sessions. As a result, we confirmed the performance and availability of the developed system with experimental user's bio-potentials.

• 농업과학연구
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• 제48권1호
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• pp.33-43
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• 2021

Along with the widespread use of pesticides in the world, concerns over human health impacts are rapidly growing. There is a large body of evidence on the relationship between the exposure to pesticides and the elevated rate of chronic diseases such as different types of cancers, diabetes, neurodegenerative disorders like Parkinson, Alzheimer, and amyotrophic lateral sclerosis (ALS), birth defects, and reproductive disorders. This research assessed the health risk of pesticide residues by the dietary intake of vegetables collected from the agro-based markets of Dhaka, Bangladesh. As some of the banned pesticides were also found in vegetable samples, they may pose a higher risk because of cheaper availability and hence the government of Bangladesh should take strong measures to control these banned pesticides. Five organo phosphorus (chlorpyrifos, parathion, ethion, acephate, fenthion) and two carbamate (carbaryl and carbofuran) pesticide residues were identified in twenty four samples of two common vegetables (tomato and brinjal). The pesticide residues ranged from below a detectable limit (< 0.01) to 0.36 mg·kg^-1. Acephate, chlorpyrifos, ethion, and carbaryl were detected in only one sample, while co-occurrence occurred twice for parathion. Continuous monitoring and strict regulation should be enforced regarding the control of pesticide residues in fresh vegetables and other food commodities in Bangladesh.