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Diagnosis and treatment in Charcot-Marie-Tooth disease (샤르코-마리-투스 질환의 진단 및 치료)

  • Kim, Sang-Beom;Park, Kee Duk;Choi, Byung-Ok
    • Annals of Clinical Neurophysiology
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    • v.7 no.2
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    • pp.65-74
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    • 2005
  • Charcot-Marie-Tooth (CMT) disease was described by Charcot and Marie in France and, independently, by Tooth in England in 1886. CMT is the most common form of inherited motor and sensory neuropathy, and is a genetically heterogeneous disorder of the peripheral nervous system. Therefore, many genes have been identified as CMT-causative genes. Traditionally, subclassification of CMT have been divided into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). Recently, intermediate type (CMT-Int) with NCVs between CMT1 and CMT2 is considered as a CMT type. There are several related peripheral neuropathies, such as $D{\acute{e}}j{\acute{e}}rine$-Sottas neuropathy (DSN), congenital hypomyelination (CH), hereditary neuropathy with liability to pressure palsies (HNPP) and giant axonal neuropathy (GAN). Great advances have been made in understanding the molecular basis of CMT, and 17 distinct genetic causes of CMT have been identified. The number of newly discovered mutations and identified genetic loci is rapidly increasing, and this expanding list has proved challenging for physicians trying to keep up with the field. Identifying the genetic cause of inherited neuropathies is often important to determine at risk family members as well as diagnose the patient. In addition, the encouraging studies have been published on rational potential therapies for the CMT1A. Now, we develop a model of how the various genes may interact in the pathogenesis of CMT disorder.

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Wind profile management and blockage assessment for a new 12-fan Wall of Wind facility at FIU

  • Aly, Aly Mousaad;Chowdhury, Arindam Gan;Bitsuamlak, Girma
    • Wind and Structures
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    • v.14 no.4
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    • pp.285-300
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    • 2011
  • Researchers at the International Hurricane Research Center (IHRC), Florida International University (FIU), are working in stages on the construction of a large state-of-the-art Wall of Wind (WoW) facility to support research in the area of Wind Engineering. In this paper, the challenges of simulating hurricane winds for the WoW are presented and investigated based on a scale model study. Three wind profiles were simulated using airfoils, and/or adjustable planks mechanism with and without grids. Evaluations of flow characteristics were performed in order to enhance the WoW's flow simulation capabilities. Characteristics of the simulated wind fields are compared to the results obtained from a study using computational fluid dynamics (CFD) and also validated via pressure measurements on small-scale models of the Silsoe cube building. Optimal scale of the test model and its optimal distance from the WoW contraction exit are determined - which are two important aspects for testing using an open jet facility such as the WoW. The main objective of this study is to further the understanding of the WoW capabilities and the characteristics of its test section by means of intensive tests and validations at small scale in order to apply this knowledge to the design of the full-scale WoW and for future wind engineering testing.

A proposed technique for determining aerodynamic pressures on residential homes

  • Fu, Tuan-Chun;Aly, Aly Mousaad;Chowdhury, Arindam Gan;Bitsuamlak, Girma;Yeo, DongHun;Simiu, Emil
    • Wind and Structures
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    • v.15 no.1
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    • pp.27-41
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    • 2012
  • Wind loads on low-rise buildings in general and residential homes in particular can differ significantly depending upon the laboratory in which they were measured. The differences are due in large part to inadequate simulations of the low-frequency content of atmospheric velocity fluctuations in the laboratory and to the small scale of the models used for the measurements. The imperfect spatial coherence of the low frequency velocity fluctuations results in reductions of the overall wind effects with respect to the case of perfectly coherent flows. For large buildings those reductions are significant. However, for buildings with sufficiently small dimensions (e.g., residential homes) the reductions are relatively small. A technique is proposed for simulating the effect of low-frequency flow fluctuations on such buildings more effectively from the point of view of testing accuracy and repeatability than is currently the case. Experimental results are presented that validate the proposed technique. The technique eliminates a major cause of discrepancies among measurements conducted in different laboratories. In addition, the technique allows the use of considerably larger model scales than are possible in conventional testing. This makes it possible to model architectural details, and improves Reynolds number similarity. The technique is applicable to wind tunnels and large scale open jet facilities, and can help to standardize flow simulations for testing residential homes as well as significantly improving testing accuracy and repeatability. The work reported in this paper is a first step in developing the proposed technique. Additional tests are planned to further refine the technique and test the range of its applicability.

A literature study on Bromidrosis (腋격의 病因.病機.治療에 對한 文獻的 考察)

  • Kim, Jong-sung;Kim, Kyung-joon
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.13 no.1
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    • pp.157-184
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    • 2000
  • Bromidrosis is a disease which is also called huchou[호격] in the oriental medicine. This disease produces an odor and a small amount of sweat from the axillary area. Prognosis of the disease could possibly get better with age. This can be explained by the fact that its symptoms begin usually when a person reaches puberty and as the person gets old, the symptoms reduce or disappear even when specific treatments are not applied. Although the disease is not lethal, many patients have difficulties in leading a normal and comfortable life, physically and psychologically with this in mind and some osbservations gained from the literature studies on the causes,symptoms, and treatments, I embarked on a study aimed at finding out the therapies. My study on the literature has produced the following results ; 1. In the oriental medicine, hu-chou[호격] means the characteric smell or odor produced from the fox. 2. The literature studies point out two causes in the oriental medicine. One is natural. The other is acquired. Acquired cases have the following causes: shi-re-nei-yun[濕熱內蘊]. xie-뱌-bu-huo[血氣不和], gan-qi-yu-jie[肝氣鬱結]. 3 There are two therapies in the oriental medicine. One is internal and the other external. out of the two, external therapies were mainly used according to the literature studies that I have consulted. 4. According to my study, the most frequently used herbal medicine was Alunitum(明 礬)(45 times), followed by Minium(密陀僧)(39 times) and Moschus(?香)(21 times respectively). 5. My study also found that bian-zheng[辨證] was not .rarely listed on old literatures. Therefore, old literatures did not clearly show how to root out the disease. They only taught now to alleviate the symptoms and remove the characteric odor.

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Fed-batch Cultivation of Escherichia coli YK537 (pAET-8) for Production of phoA Promoter-controlled Human Epidermal Growth Factor

  • Wang Yonggang;Du Peng;Gan Renbao;Li Zhimin;Ye Qin
    • Biotechnology and Bioprocess Engineering:BBE
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    • v.10 no.2
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    • pp.149-154
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    • 2005
  • Secretion of the expressed heterologous proteins can reduce the stress to the host cells and is beneficial to their recovery and purification. In this study, fed-batch cultures of Escherichia coli YK537 (pAET-8) were conducted in a 5-L fermentor for the secretory production of human epidermal growth factor (hEGF) whose expression was under the control of alkaline phosphatase promoter. The effects of feeding of glucose and complex nitrogen sources on hEGF production were investigated. When the fed-batch culture was conducted in a chemically de-fined medium, the cell density was 9.68 g/L and the secreted hEGF was 44.7 mg/L in a period of 60 h. When a complex medium was used and glucose was added in pH-stat mode, the secreted hEGF was improved to 345 mg/L. When the culture was fed with glucose at a constant specific rate of $0.25\;gg^{-1}h^{-1}$, hEGF reached 514 mg/L. The effects of adding a solution containing yeast extract and tryptone were further studied. Different rate of the nitrogen source feeding resulted in different levels of phosphate and acetic acid formation, thus affected hEGF expression. At the optimal feeding rate, hEGF production achieved 686 mg/L.

Microplastic Management for Preventing Risk of Persistent/Bioaccumulative Substance (잔류성.생물축적성 물질 피해저감을 위한 미세플라스틱(Microplastic) 관리방안)

  • Park, Jeong-Gue;Gan, Sun-Yeong
    • Journal of Environmental Policy
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    • v.13 no.2
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    • pp.65-98
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    • 2014
  • Plastics of the marine environment are broken gradually down into smaller particles by chemical weathering, called "microplastic". Microplastics absorb organic pollutants that are persistent bioaccumulative substances. If marine animals ingested microplastic added to contaminant, it will lead to a bioaccumation through the food web. It eventually destroy health of marine environment and is harmful to marine top predators including humans. Also, Microplastics can impact marine animals by leaching the endocrine disruptor in microplastic itself as well as playing an adsorbent role of organic pollutants. Persistent and bioaccumulative substances in Korea have been regulated in terms of chemical risk but existing regulations largely have been limited in land-based source management of microplastic. Thus, the harmful impact will be increased whether the microplastics absorbed contaminants. To prevent risk of persistent bioaccumulative substances, this study suggests the following: (1) the strict management of microplastic by designating the hazardous substances, (2) expand the use of biodegradable plastic, (3) the effort for reuse and recycle, (4) the expand of microplastic clean-up programs.

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A Study of Architectural and Design Elements of Secondary Shirines in Traditional Buddhist Temples in Korea (한국 전통사찰에 있어서 부불전의 건축요소와 의장적 특성에 관한 연구)

  • Cho, Jeong-Sik
    • Journal of the Korean Institute of Landscape Architecture
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    • v.38 no.5
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    • pp.102-112
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    • 2010
  • The purpose of this study is to identify the architectural design elements of secondary shrines in traditional Buddhist temples in Korea. For this study, a survey of six kinds of secondary shrines (Myemgbujem, Yeongsanjeon, Eungjinjem, Yaksajeoun, Gwaneumjeon, Mireukjeon), according to specified design elements(building sizes, roof styles, Gongpo, Dancheong, shape of Datjip), was conducted. The results of this study are summarized as follows; Myeongbujem is built in every temples(25 examples), most having simila1ities in sizes and architectural elements(roof, Gongpo structure, Dancheong, Datjip). The majority of Yemgsanjeon(l4 examples) and Eungjinjem shrines(l5 examples) are mostly 3-Gan sized buildings, having a Matbaejibung and Ikkmg structural system. Gwaneumjeon, on the other hands, has 1he most elaborate architectural elements. Among 12 cases, 8 buildings have Paljakjibung, 9 buildings have Silk-Dancheong in innerspace, and Datjip are included in six buildings. Yaksajeon shrines are small in mnnber, but most of these shrines have a Dapo structure, and, despite of their size, are designated as cultural properties.

Antibacterial Mode of Action of Cinnamomum verum Bark Essential Oil, Alone and in Combination with Piperacillin, Against a Multi-Drug-Resistant Escherichia coli Strain

  • Yap, Polly Soo Xi;Krishnan, Thiba;Chan, Kok-Gan;Lim, Swee Hua Erin
    • Journal of Microbiology and Biotechnology
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    • v.25 no.8
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    • pp.1299-1306
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    • 2015
  • This study aims to investigate the mechanism of action of the cinnamon bark essential oil (CB), when used singly and also in combination with piperacillin, for its antimicrobial and synergistic activity against beta-lactamase TEM-1 plasmid-conferred Escherichia coli J53 R1. Viable count of bacteria for this combination of essential oil and antibiotic showed a complete killing profile at 20 h and further confirmed its synergistic effect by reducing the bacteria cell numbers. Analysis on the stability of treated cultures for cell membrane permeability by CB when tested against sodium dodecyl sulfate revealed that the bacterial cell membrane was disrupted by the essential oil. Scanning electron microscopy observation and bacterial surface charge measurement also revealed that CB causes irreversible membrane damage and reduces the bacterial surface charge. In addition, bioluminescence expression of Escherichia coli [pSB1075] and E. coli [pSB401] by CB showed reduction, indicating the possibility of the presence of quorum sensing (QS) inhibitors. Gas-chromatography and mass spectrometry of the essential oil of Cinnamomum verum showed that trans-cinnamaldehyde (72.81%), benzyl alcohol (12.5%), and eugenol (6.57%) were the major components in the essential oil. From this study, CB has the potential to reverse E. coli J53 R1 resistance to piperacillin through two pathways; modification in the permeability of the outer membrane or bacterial QS inhibition.

Evolution and Mineralizations in the Ockcheon Geosynclinal Zone (옥천지향사대(沃川地向斜帶)의 진화(進化)와 광화작용(鑛化作用))

  • Yun, Suckew
    • Economic and Environmental Geology
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    • v.4 no.2
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    • pp.77-90
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    • 1971
  • About four hundred deposits of iron, talc, fluorite, tungsten, molybdenum, lead, zinc and other polymetallic mineral deposits were plotted on the Ore Distribution Map of the Ockcheon Geosynclinal Area. These mineral deposits plotted on the map can be divided into the several metallogenic zones by the consideration of their geologic background including the sedimentary and tectonic cycles and the igneous activities in the geosynclinal evolution, as follows: a. Chungju iron and talc zones. b. Cheong-san copper bearing iron sulphide zone c. Kumsan-Muju fluorite-polymetallic zones. d. Cheong-an Puyong and Ein Suckseong gold zone e. Hwang-gan Seolcheon and Sangju gold zones. Chungju iron zone originated in the iron bed in the Kemyongsan Series corresponding to the Pre-Ockcheon Cycle of evolution history. In early period of the Ockcheon Cycle, Hyangsanri quartzite and Munjuri phyllitic formation corresponding to the lower terrigenous sequence were not mineralized while the next sequence of the Samsungsan basic igneous-metamorphic formation and the Changri limestone formation were mineralized by the copper bearing iron sulphide and the fluorite-polymetallic deposits respectively. Two generations of the gold zones are recognized. The earlier generation distributes directionaly in the outside of the Ockcheon sedimentary belt was followed by the earlier grantitic invasion of Jurasic in age, while the later generation scatters at random which was related to the nondirectional Cretaceous granitic intrusion of the Post-Ockcheon Cycle. Conclusively speaking, it was disclosed that the endogenic mineralization in the Ockcheon geosyn clinal zone was not conspicuous in its inner sedimentary belt except its limestone area but in its outer peripheral granitic or gneissic zones, and the related igneous activities occured in the Post-Ockcheon Cycle of evolution history.

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A Study on the Synthetic ECG Generation for User Recognition (사용자 인식을 위한 가상 심전도 신호 생성 기술에 관한 연구)

  • Kim, Min Gu;Kim, Jin Su;Pan, Sung Bum
    • Smart Media Journal
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    • v.8 no.4
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    • pp.33-37
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    • 2019
  • Because the ECG signals are time-series data acquired as time elapses, it is important to obtain comparative data the same in size as the enrolled data every time. This paper suggests a network model of GAN (Generative Adversarial Networks) based on an auxiliary classifier to generate synthetic ECG signals which may address the different data size issues. The Cosine similarity and Cross-correlation are used to examine the similarity of synthetic ECG signals. The analysis shows that the Average Cosine similarity was 0.991 and the Average Euclidean distance similarity based on cross-correlation was 0.25: such results indicate that data size difference issue can be resolved while the generated synthetic ECG signals, similar to real ECG signals, can create synthetic data even when the registered data are not the same as the comparative data in size.