• 제목/요약/키워드: amyotrophic lateral sclerosis

검색결과 122건 처리시간 0.029초

Coenzyme Q10: a progress towards the treatment of neurodegenerative disease

  • Kumar, Peeyush;Kumar, Pramod;Ram, Alpana;Kuma, Mithilesh;Kumar, Rajeev
    • Advances in Traditional Medicine
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    • 제10권4호
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    • pp.239-253
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    • 2010
  • Coenzyme $Q_{10}$ ($CoQ_{10}$, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain (electron transport chain) with antioxidant properties. In view of the involvement of $CoQ_{10}$ in oxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone would be expected to contribute to disease pathophysiology by causing a failure in energy metabolism and antioxidant status. Indeed, a deficit in $CoQ_{10}$ status has been determined in a number of neuromuscular and neurodegenerative disorders. Primary disorders of $CoQ_{10}$ biosynthesis are potentially treatable conditions and therefore a high degree of clinical awareness about this condition is essential. A secondary loss of $CoQ_{10}$ status following HMG-CoA reductase inhibitor (statins) treatment has been implicated in the pathophysiology of the myotoxicity associated with this pharmacotherapy. $CoQ_{10}$ and its analogue, idebenone, have been widely used in the treatment of neurodegenerative and neuromuscular disorders. These compounds could potentially play a role in the treatment of mitochondrial disorders, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of $CoQ_{10}$, as well as the rationale and the role in clinical practice of $CoQ_{10}$ supplementation in different neurological diseases, from primary $CoQ_{10}$ deficiency to neurodegenerative disorders. These will help in future for treatment of patients suffering from neurodegenerative disease.

A Case Report on the Use of Korean Medicine Treatment for a Patient with Dysarthria Caused by Progressive Bulbar Palsy (PBP)

  • Ahn, Jae Hyun;Kim, Sung Yoon;Park, Jun Hyeong;Cho, Jeongjae;Choi, Do Young;Lee, Seung Hoon;Lee, Jae Dong
    • Journal of Acupuncture Research
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    • 제34권1호
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    • pp.49-58
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    • 2017
  • Objectives : To introduce a rare case of a patient with Progressive Bulbar Palsy (PBP) and suggest the possibility of treatment using electroacupuncture and Korean Medicine. Methods : A 61-year-old man with PBP, complaining of dysarthria, was treated with electroacupuncture and Korean Medicine, from June 16 to July 10, 2015. Improvements in symptoms were measured using the speech mechanism screening test (SMST), measurement of tongue and orbicularis oris motility, and speech handicap index (SHI). Results : The scores of SMST, motility of tongue and orbicularis oris showed a tendency for gradual improvement with 25 days of Korean Medicine Treatment after admission, but conversation was still impossible. In SHI scores, one point increased in the speech domain and one point decreased in the psycho-social domain, and three points increased in the other domain. Conclusion : Electroacupuncture and Korean Medicine Treatment improved dysarthria caused by PBP, but not completely. Korean Medicine Treatment seems effective in the management of accompanying symptoms such as black hairy tongue, dry mouth, and general condition. The symptoms of PBP are similar to those of amyotrophic lateral sclerosis and there is controversy regarding the classification of PBP. The most important aspect of treating a patient with PBP is an early diagnosis and devising appropriate rehabilitation strategies.

Glutamate 매개 흥분성 신경독성에 대한 봉독의 NSC-34 신경세포사멸 억제 효과 (Effect of Bee Venom on Glutamate-mediated Excitotoxicity in NSC-34 Motor Neuronal Cells)

  • 이상민;최선미;정소영;양은진
    • 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.

가정용 인공호흡기 적용 환자의 가정간호서비스 이용실태 (A Survey of Hospital-Based Home Healthcare Utilization in Patients using Home Mechanical Ventilator)

  • 이미경;송종례;오은경;윤영미
    • 가정간호학회지
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    • 제24권2호
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    • pp.210-220
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    • 2017
  • Purpose: The study aimed to describe the utilization of home healthcare in patients using home mechanical ventilator(HMV) Method: A descriptive cross-sectional design was used in this study. A Questionnaires were sent to nation wide home healthcare agencies to assess their utilization status of home healthcare. A convenience sample of 158 patients data was reviewed. Result: A total of 88(55.7%) men with the mean age of 51.94(${\pm}19.52$) years were included in the study. Approximately 55.1% of patients at the outpatient department were referred to the home healthcare services after discharge. The underlying diseases were as follows : 129 amyotrophic lateral sclerosis and 27 muscular dystrophies. A total of 155 patients have invasive HMV. Efficient home healthcare nursing activities provided by a highly skilled home healthcare advanced practice nurses(HHCAPN) were tracheotomy and gastrostomy tube management and urinary catheterization. The average frequency of home visit for one patient was 2.52times per month. The duration of home healthcare utilization with >1 year was 82.9%. HHCAPNs have limited knowledge and skill for HMV. Conclusion: The government support is required to provide sufficient home healthcare services to the patients discharged with HMV. HHCAPNs should be properly educated on the effective HMV care.

생체전위를 이용한 중증 운동장애자들을 위한 컴퓨터 접근제어장치 설계 (Design of Computer Access Devices for Severly Motor-disability Using Bio-potentials)

  • 정성재;김명동;박찬원;김일환
    • 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.

중증 언어장애인을 위한 의사소통 시스템 (Communication Support System for Person with Language Disabilities)

  • 홍승욱;박수현
    • 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.

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26-Week Repeated Dose Oral Toxicity Study of KCHO-1 in Sprague-Dawley Rats

  • Yang, Muhack;Lee, Seongjin;Wang, Tingting;Cha, Eunhye;Jang, Jongwon;Kim, Dongwoung;Song, Bong-Keun;Son, Ilhong;Kim, Joonyup;Kang, Hyung Won;Kim, Sungchul
    • 대한약침학회지
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    • 제22권3호
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    • pp.192-199
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    • 2019
  • Objectives: KCHO-1(Mecasin), also called Gamijakyakgamchobuja-tang originally, is a combination of some traditional herbal medicines in East Asia. This medicine has been used mainly for alleviating neuropathic pains for centuries in Korean traditional medicine. KCHO-1 was developed to treat pain, joint contracture and muscular weakness in patients with amyotrophic lateral sclerosis. This study was carried out to investigate the chronic toxicity of KCHO-1 oral administration in rats for 26 weeks. Methods: Sprague-Dawely rats were divided into four groups and 10 rats were placed in the control group and the high-dose group, respectively. Group 1 was the control group and the remaining groups were the experimental groups. In the oral toxicity study, 500 mg/kg, 1,000 mg/kg, and 2,000 mg/kg of KCHO-1 were administered to the experimental group, and 10 ml/kg of sterile distilled water was administered to the control group. Survival rate, body weight, feed intake, clinical signs, and visual findings were examined. Urinalysis, ophthalmologic examination, necropsy, organ weight, hematologic examination, blood chemical examination and histopathologic examination were performed. Results: Mortality and toxicological lesions associated with the administration of test substance were not observed in all groups. Conclusion: NOAEL(No observed adverse effect level) of KCHO-1 is higher than 2000 mg/kg/day. And, the above findings suggest that treatment with KCHO-1 is relatively safe.

Probabilistic exposure assessment, a risk-based sampling plan and food safety performance evaluation of common vegetables (tomato and brinjal) in Bangladesh

  • Mazumder, Mohammad Nurun-Nabi;Bo, Aung Bo;Shin, Seung Chul;Jacxsens, Liesbeth;Akter, Tahmina;Bir, Md. Shahidul Haque;Aktar, Most Mohshina;Rahman, Md. Habibur;WeiQiang, Jia;Park, Kee Woong
    • 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.

NLRP3 인플라마좀 작용 기전 및 신경 질환에서의 역할 (NLRP3 Inflammasome in Neuroinflammatory Disorders)

  • 김지희;김영희
    • 생명과학회지
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    • 제31권2호
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    • pp.237-247
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    • 2021
  • 신경염증(neuroinflammation)은 여러 신경 질환의 원인 인자로 확인되고있다. 중추 신경계에 발현되는 단백질 복합체인 NLRP3 인플라마좀은 미생물, 응집되고 잘못 접힌 단백질, ATP와 같은 광범위한 외인성 및 내인성 자극에 의해 감지되고 캐스페이즈-1(capase-1)을 활성화할 수 있다. 활성을 띠는 캐스페이즈-1은 IL-1b와 IL-18과 같은 염증성 사이토카인(pro-inflammatory cytokine)을 활성화시키고 급속한 세포사멸(파이롭토시스, pyroptosis)를 야기한다. IL-1b와 IL-18, 그리고 파이롭토시스를 통해 분비된 DAMPs은 다양한 신호 전달 경로를 통해 신경염증 반응을 유도하여 신경 손상을 유발한다. 따라서 NLRP3 인플라마좀은 신경염증으로 인한 여러 가지 신경질환 발병에 중요한 역할을 할 것으로 여겨진다. 본 리뷰 에서는 NLRP3 인플라마좀의 구조와 활성화에 대해 간략히 알아 보고 다양한 형태의 신경 질환에서 NLRP3 인플라마좀의 역할에 대해 논의하고자 한다.

인간 내생 레트로바이러스(Human Endogenous Retrovirus, HERV)의 염증반응 조절 기작 (Mechanism of Human Endogenous Retrovirus (HERV) in Inflammatory Response)

  • 고은지;차희재
    • 생명과학회지
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    • 제31권8호
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    • pp.771-777
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    • 2021
  • 인간 내생 레트로바이러스(Human Endogenous Restrovirus, HERV)는 수백만년전 인간의 유전체에 삽입되었으며 이후 오랜 세월을 거치며 재조합, 결실 및 돌연변이 등 여러 원인에 의해 더 이상 활성화된 바이러스로 역할을 하지 못하고 감염되지 않는다. 하지만 HERV는 최근 연구들은 HERV 유래 인자들이 실제 생리현상 및 암을 비롯한 특정 질환에 관여 하고 있다는 것을 보여 주었다. HERV와 관련된 여러가지 생리 현상 중 염증반응에 초점을 맞추어 고찰해 볼 필요가 있다. HERV는 류마티스, 다발성 경화증, 근위축성 측삭경화증, 쇼그렌 증후군 같은 자가면역질환을 비롯한 여러 염증질환에 직접적으로 관여하는 것으로 보고 되고 있다. HERV의 염증 조절 기작으로는 HERV 유래 인자들이 비특이적 선천성 면역과정을 유발할 가능성과 HERV 유래의 RNA와 단백질이 특정 수용체를 통해 선택적 신호전달기작을 유발할 가능성을 고려 할 수 있다. 하지만 어떠한 방식으로 잠재되어 있던 HERV가 염증반응에서 활성화 되는지 또한 HERV와 관여된 인자들과 신호기작들이 어떠한 것들이 있는지 등 HERV의 인자들이 염증반응을 조절하는 기작에는 아직 많은 것들이 밝혀지지 않아 질병 발병에 대한 연구에 어려움이 있는 실정이다. 본 리뷰에서는 HERV 관련 자가 면역질환을 소개하고 염증반응 조절 기작에 관한 HERV의 분자수준에서의 작용 메커니즘을 제안 하고자 한다.