• Biomolecules & Therapeutics
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• 제26권3호
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• pp.225-241
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• 2018

Taurine is an abundant, ${\beta}-amino$ acid with diverse cytoprotective activity. In some species, taurine is an essential nutrient but in man it is considered a semi-essential nutrient, although cells lacking taurine show major pathology. These findings have spurred interest in the potential use of taurine as a therapeutic agent. The discovery that taurine is an effective therapy against congestive heart failure led to the study of taurine as a therapeutic agent against other disease conditions. Today, taurine has been approved for the treatment of congestive heart failure in Japan and shows promise in the treatment of several other diseases. The present review summarizes studies supporting a role of taurine in the treatment of diseases of muscle, the central nervous system, and the cardiovascular system. In addition, taurine is extremely effective in the treatment of the mitochondrial disease, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), and offers a new approach for the treatment of metabolic diseases, such as diabetes, and inflammatory diseases, such as arthritis. The review also addresses the functions of taurine (regulation of antioxidation, energy metabolism, gene expression, ER stress, neuromodulation, quality control and calcium homeostasis) underlying these therapeutic actions.

• 한국가금학회지
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• 제38권1호
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• pp.45-50
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• 2011

본 증례는 국내의 산란계 농장에서 사육 중이던 39일령 산란계 중추계군에서 신경 증상을 동반한 폐사를 나타내며, 부검 시 폐를 포함한 뇌, 간, 신장 등의 실질장기에서 직경 1~5 mm 크기의 황백색 결절이 다수 관찰되었다. 병리조직학적으로 다발성의 육아종성 병변이 폐, 뇌, 신장, 비장 등에서 관찰되었으며, 특히 폐의 결절 형성 및 건락성 육아종성 병변이 심한 정도로 나타나 호흡기를 통해 1차 감염이 이루어진 것을 확인할 수 있었다. 육아종성 결절을 SDA에 배양하여 집락의 색을 확인하고, 균사체의 Giemsa 염색 및 조직학적 병변상에서 PAS 염색 결과를 통해 Aspergillus 속 곰팡이인 것으로 확인할 수 있었고, 배양된 균사에 대한 유전학적 검사 결과 Aspergillus flavus로 동정되었다. A. flavus 균사는 폐, 뇌, 신장 및 선위에서 병변 중심부 및 변연부 모두에서 관찰되었고, 다핵 거대세포 및 섬유아세포가 염증세포들과 함께 둘러싸고 있는 것이 확인되었다. 특히 대뇌에서의 육아종성 병변 및 그로 인한 신경 증상이 임상 증상으로 발현된 것이 본 증례의 특징적인 소견이다.

• Journal of Preventive Medicine and Public Health
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• 제2권1호
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• pp.61-76
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• 1969

A review has been made of mortality trends in Korea from 1958 to 1967 analyzing the data by sex, age and cause of death. The crude death rates and age specific death rates were estimated by the model of N. Keyfitz life table which had been developed by the data of the 1960's national census. The cause specific death rates shown in this article are based on the following: all deaths occurring in the death-registration are expressed as a numberator, while the denominator was estimated from the regular national census data by interpolation method. It is estimated that only an average of about 40% of deaths which occurred during a year were registered during 1958 to 1967. The validity and the reliability of the diagnosis of causes of death seem to be extremely poor in this country. Therefore the cause specific death rates in this article are aimed to reveal trends of causes of registered death ana not for the actual level of death rates. For 10 years very interesing mortality trends were observed : 1. The trend in the crude death rates was downward slowly. 2. The estimated death rate for the infant in 1960 was still high up to 100 per 1,000. 3. The rates for mortality attributed to such infectious diseases as pneumonia, bronchitis, gastroenteritis and measles decreased an average 40-60%. 4. The death rates for over-all tuberculosis decreased only 9.8%. 90% of the decrease was contributed by those in the less-than-15 year age group. 5. The death rates for chronic diseases, such as vascular diseases affecting the central nervous system, malignant neoplasm, major heart diseases and all accidents rose about 40-60%. 6. The rank order of the 10 leading causes of death showed large changes over the years, except for pneumonia and tuberculosis which occupyed 1st and 2nd places respectively. Vascular diseases affecting the central nervous system moved from 5th to 3rd place and malignant neoplasm from 6th to 4th place, The major heart diseases moved from 10th to 6th place and all accidents from 10th to 7th place. On tile other hand, gastroenteritis moved from 3rd to 5th place and influenja from 4th to 8th place.

• BMB Reports
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• 제49권9호
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• pp.474-487
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• 2016

Itch is one of the most distressing sensations that substantially impair quality of life. It is a cardinal symptom of many skin diseases and is also caused by a variety of systemic disorders. Unfortunately, currently available itch medications are ineffective in many chronic itch conditions, and they often cause undesirable side effects. To develop novel therapeutic strategies, it is essential to identify primary afferent neurons that selectively respond to itch mediators as well as the central nervous system components that process the sensation of itch and initiate behavioral responses. This review summarizes recent progress in the study of itch, focusing on itch-selective receptors, signaling molecules, neuronal pathways from the primary sensory neurons to the brain, and potential decoding mechanisms based on which itch is distinguished from pain.

• Natural Product Sciences
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• 제13권3호
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• pp.268-271
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• 2007

Glutamate-induced oxidative injury contributes to neuronal degeneration in many central nervous system (CNS) diseases, such as epilepsy and ischemia. Bioassay-guided fractionation of the MeOH extract of the seeds of Alpinia katsumadai Hayata (Zingiberaceae) furnished three phenolic compounds, alpinetin (1), pinocembrin (2), and (+)-catechin (3). Compounds 2 and 3 showed the potent neuroprotective effects on glutamate-induced neurotoxicity and reactive oxygen species (ROS) generation in the mouse hippocampal HT22 cells. In addition, Compounds 2 and 3 showed significant DPPH free radical scavenging effect. These results suggest that compounds 2 and 3 could be the effective candidates for the treatment of ROS-related neurological diseases.

• Journal of mucopolysaccharidosis and rare diseases
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• 제3권1호
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• pp.1-8
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• 2017

Mucopolysaccharidosis (MPS) type III, or Sanfilippo syndrome is a rare autosomal recessive lysosomal storage disorder. It is caused by a deficiency of one of four enzymes involved in the degradation of the glycosaminoglycan (GAG) heparan sulfate. The resultant cellular accumulation of heparan sulfate causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme: MPS IIIA, MPS IIIB, MPS IIIC and MPS IIID. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system (CNS). Main purpose of the treatment for MPS III is to prevent neurologic deterioration. However, conventional enzyme replacement therapy has a limitation due to inability to cross the blood-brain barrier. Several experimental treatment options for MPS III are being developed.

• Molecular & Cellular Toxicology
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• 제3권2호
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• pp.77-84
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• 2007

Millions of individuals worldwide are currently afflicted with neurodegenerative disorders such as Parkinson's disease and multiple sclerosis which are caused by the death of specific types of specialized cells in the Central Nervous System (CNS). Recently, Neural Stem Cells (NSCs) are able to replace these dead cells with new functional cells, thereby providing a cure for devastating neural diseases. The clinical use of neural stem cells (NSCs) for the treatment of neurological diseases requires overcoming the scarcity of the initial in vivo NSC population. Thus, we developed a novel 3-dimentional cellular automata model for optimum production of neural stem cells and their derivatives in large scale to treat neurodegenerative disorder patients.

• BMB Reports
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• 제53권1호
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• pp.28-34
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• 2020

Sphingolipids are ubiquitous building blocks of eukaryotic cell membranes that function as signaling molecules for regulating a diverse range of cellular processes, including cell proliferation, growth, survival, immune-cell trafficking, vascular and epithelial integrity, and inflammation. Recently, several studies have highlighted the pivotal role of sphingolipids in neuroinflammatory regulation. Sphingolipids have multiple functions, including induction of the expression of various inflammatory mediators and regulation of neuroinflammation by directly effecting the cells of the central nervous system. Accumulating evidence points to sphingolipid engagement in neuroinflammatory disorders, including Alzheimer's and Parkinson's diseases. Abnormal sphingolipid alterations, which involves an increase in ceramide and a decrease in sphingosine kinase, are observed during neuroinflammatory disease. These trends are observed early during disease development, and thus highlight the potential of sphingolipids as a new therapeutic and diagnostic target for neuroinflammatory diseases.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.37-41
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• 2018

Mucopolysaccharidosis III (MPS III, Sanfilippo syndrome) is a rare autosomal recessive disease caused by a deficiency of one of four enzymes involved in the degradation of glycosaminoglycan (GAG). The resultant cellular accumulation of GAG causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system. A number of genetic and biochemical diagnostic methods have been developed. However, there is no effective therapy available for any form of MPS III, with treatment currently limited to clinical management of neurological symptoms. Main purpose of the treatment for MPS III is to prevent neurologic deterioration. Because conventional intravenous enzyme replacement therapy (ERT) has a limitation due to inability to cross the blood-brain barrier, several innovative therapeutic approaches for MPS III are being developed. This review covers the currently developing new therapeutic options for MPS III including high dose ERT, substrate reduction therapy, intrathecal or intraventricular ERT, fusion protein delivery using bioengineering technology, and gene therapy.

• Clinical and Experimental Pediatrics
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• 제64권3호
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• pp.103-110
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• 2021

Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.