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Two cases of spinal muscular atrophy type 1 with extensive involvement of sensory nerves (광범위한 감각신경 침범을 동반한 척수성 근위축증 2예)

  • Lee, Ran;Chung, Sochung;Koh, Sung-Eun;Lee, In Kyu;Lee, Jongmin
    • Clinical and Experimental Pediatrics
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    • v.51 no.12
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    • pp.1350-1354
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    • 2008
  • Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by diffuse proximal and distal weakness due to deletion of the survival motor neuron (SMN) gene localized on chromosome 5 (5q11.2-13.3). SMA has been considered as a pure lower motor neuron disorder, and a definitive diagnosis can be established by molecular genetic testing. Here, we describe two patients with severe hypotonia and frequent aspirations at early infancy. Nerve conduction studies showed more extensive sensory involvement in these patients diagnosed to have SMA by genetic study than in classical cases of SMA. To the best of our knowledge, this is the first report of SMA Type 1 with sensory nerve involvement in Korea.

Thermal-Structural Coupled Field Analysis of the Circumferential Pressing Type Brake Disc (원주가압형 브레이크 디스크의 열-구조 연성해석)

  • Kim, Hyeong-Hoon;Lee, Seong-Wook;Han, Dong-Seop;Han, Geun-Jo
    • Transactions of the Korean Society of Automotive Engineers
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    • v.16 no.4
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    • pp.69-74
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    • 2008
  • The heat generated by the brake system of vehicles results in reduction of friction force on the brake surface and vibration during a braking. To solve these problems, extensive research for the brake shape has been conducted such as drilling cooling holes on the brake disc, accommodating ventilated holes and etc. In this study, we suggest the circumferential pressing type brake disc in order to improve its cooling performance. In order to compare the cooling-down efficiency between the conventional side-pressing type and the circumferential-pressing type, we adopted the FMVSS 105-77 as thermal analysis conditions and This newly proposed concept has been verified using Thermal-structure Coupled Field Analysis along with comparative analysis with the existing ventilated disk.

Heterologous Expression of Hybrid Type II Polyketide Synthase System in Streptomyces Species

  • Kim, Chang-Young;Park, Hyun-Joo;Kim, Eung-Soo
    • Journal of Microbiology and Biotechnology
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    • v.13 no.5
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    • pp.819-822
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    • 2003
  • Polyketides are an extensive class of secondary metabolites with diverse molecular structures and biological activities. A plasmid-based minimal polyketide synthase (PKS) expression cassette was constructed using a subset of actinorhodin (act) biosynthetic genes (actI-orfl, actI-orf2, actI-orf3, actIII, actⅦ, and actIV) from Streptomyces coelicolor, which specify the construction of an orange-fluorescent anthraquinone product aloesaponarin II, a type II polyketide compound derived from one acetyl coenzyme A and 7 malonyl coenzyme A extender units. This system was designed as an indicator pathway in S. parvulus to generate a hybrid type II polyketide compound via gene-specific replacement. The act ${\beta}-ketoacyl$ synthase unit (actI-orfl and actI-orf2) in the expression cassette was specifically replaced with oxytetracycline ${\beta}-ketoacyl$ synthase otcY-orfl and otcY-orf2). This plasmid-based hybrid PKS cassette generated a novel orange-fluorescent compound structurally different from aloesaponarin II in both S. lividans and S. parvulus. In addition, several additional distinctive blue-fluorescent compounds were detected, when this hybrid PKS cassette was expressed in S. coelicolor B78 (actI-orf2 mutant), implying that the expression of plasmid-based hybrid PKS cassette in Streptomyces species should be an efficient way of generating hybrid type II polyketide compounds.

On dark matter haloes of barred disc galaxies

  • Sodi, Bernardo Cervantes;Li, Cheng;Park, Changbom;Wang, Lixin;Lin, Ye
    • The Bulletin of The Korean Astronomical Society
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    • v.39 no.2
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    • pp.56.2-56.2
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    • 2014
  • We present an extensive study of the environment of galaxies with bars in the low-redshift Uni-verse, using a volume-limited sample of over 30,000 galaxies drawn from the Sloan Digital Sky Survey, with visually-determined morphological classifications and bar identifications. We use four different statistics to quantify the environment of our galaxies: the projected two-point cross-correlation function with respect to a spectroscopic sample of reference galaxies, the background-subtracted number count of galaxies in a deep photometric sample in the vicinity of our galaxies, the overdensity of the local environment estimated at ~3 Mpc scale from the three-dimensional reconstruction of the cosmic density field of the local Universe, and the membership of our galaxies in the SDSS galaxy groups to segregate central to satellite systems. We find a weak, but significant trend for early-type galaxies with a bar to be more strongly clustered on scales from a few 100 kpc to 1 Mpc, when compared to early-type galaxies without a bar. For late-type galaxies, we find less neighbours within ~50 kpc around the barred late-types when compared to the unbarred late-types. For late-type galaxies we also detect a decrease of the bar fraction for dark matter dominated systems, and finally we find no obvious correlation between the overdensity and the fraction of barred galaxies in our sample.

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A Case of Membranoproliferative Glomerulonephritis Superimposed on Type 1 Diabetes Mellitus (제1형 당뇨병 환자에서 병발한 막증식성 사구체신염 1례)

  • Choi Hyun-Jin;Cho, Hee-Yeon;Kang Ju-Hyeong;Yang Sei-Won;Cheong Hae-Il;Choi Yong;Moon Kyung-Chul;Ha Il-Soo
    • Childhood Kidney Diseases
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    • v.9 no.1
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    • pp.97-101
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    • 2005
  • Renal diseases other than diabetic nephropathy can occur In diabetic patients. Urinary abnormalities or clinical courses inconsistent with the natural progression of diabetic nephropathy are suggestive of non-diabetic renal diseases and should lead to more extensive investigations. Presence of non-diabetic renal diseases in diabetic patients can alter the treatment plan and the prognosis. We report a 9-year-old girl who had type 1 membranoproliferatiye glomerulonephritis as well as type 1 diabetes mellitus.

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The study for developing Wind and Photovoltaic power hybrid generation system and monitoring (풍력.태양광 복합 발전 시스템 개발 및 모니터링에 관한 연구)

  • Park, Kunhyun;Kang, Chulung;Lim, Jonghwan;Park, Euijang
    • 한국신재생에너지학회:학술대회논문집
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    • 2010.06a
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    • pp.183.1-183.1
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    • 2010
  • Recently, the increased interest in environmental issues has led to extensive research for development of green energy generation systems. However, only one type of generation system may not be sufficient for stand-alone mode because it cannot cope with the irregularity of weather condition. A hybrid generation system is able to make up for the weakness of each system. In this paper, a stand-alone hybrid wind/PV system is developed that can guarantee the stable energy supply. The system is suitable for power supply under 50W, and a vertical savonius type of blade was designed and applied for the wind generation system.

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Horizontal Reheating of Aluminium Alloys for Thixoforming (Thixoforming을 위한 연주 Billet의 수평형 재가열)

  • Park S. M.;Kang C. G.
    • Proceedings of the Korean Society for Technology of Plasticity Conference
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    • 2001.05a
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    • pp.120-123
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    • 2001
  • The semi-solid casting will have a higher internal integrity, mechanical properties and dimensional accuracy than the conventional castings. This process can reduce the manufacturing costs and finished weight for critical components. The semi-solid casting are capable of greater dimensional repeatbility, this supplies considerable savings when extensive machining, salvage and scrap are key variables in the current automotive product. One of the most important factor regarding the semi-solid die casting process are the reheating method of the raw materials to the semi-solid state. Therefore, in this present work, the horizontal type induction heating system to obtain the optimal reheating conditions suitable for semi-solid die casting process was designed and manufactured. And the microstructure of reheated materials was investigated.

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Controlling Work Function of Graphene by Chemical Doping

  • Lee, Ji-A
    • Proceedings of the Korean Vacuum Society Conference
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    • 2013.02a
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    • pp.628-628
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    • 2013
  • Graphene, a single layer of graphite, has raised extensive interest in a wide scientific community for its extraordinary thermal, mechanical, electrical and other properties [1,2]. However, because of zero-band gap of graphene, it is difficult to apply for electronic applications. To overcome this problem, chemical doping is one of way to opening grahene bandgap. According to experimental results, by changing doping concentration and doping time, it is possible to control work function of graphene. We can obtain results through raman spectroscopy, UPS, Sheet resistance. Moreover, electronic properties of doped graphene were studied by making field effect transistors. We were able to control the doping concentration, dirac point of graphene and work function of graphene by formng n-type, p-type doping materials. In this research, the chemicals of diazonium salts, viologen, etc. were used for extrinsic doping.

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Empirical Design of an On and Off Type Solenoid Actuator For Valve Operation

  • Sung, Baek-Ju;Lee, Eun-Woong;Kim, Hyoung-Eui
    • KIEE International Transaction on Electrical Machinery and Energy Conversion Systems
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    • v.4B no.2
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    • pp.39-46
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    • 2004
  • Modern users demand that the on and off type solenoid actuator should be smaller, lighter in weight, lower in consumption power, and higher in response time. The complete design satisfying such requirements can be achieved when electromagnetic theories and empirical knowledge are combined. This paper presents various types of empirical coefficients essentially needed for optimal design of a solenoid actuator. The values of these empirical coefficients are obtained through extensive experiments over a great length of time for various kinds of solenoid actuators. We have developed a design program that is composed by combination of governing equations and empirical coefficients, and have also manufactured a prototype solenoid actuator based on the final results of the design program. The propriety of the design program and empirical coefficients have been proven by experiments.

Molecular Cloning, Bioinformatics Analysis and Expression Profiling of a Gene Encoding Vacuolar-type $H^+-ATP$ Synthetase (V-ATPase) c Subunit from Bombyx mori

  • Lu, Peng;Chen, Keping;Yao, Qin;Yang, Hua-Jun
    • International Journal of Industrial Entomology and Biomaterials
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    • v.15 no.2
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    • pp.115-122
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    • 2007
  • As the genome of B.mori is available in GenBank and the EST database of B.mori is expanding, identification of novel genes of B.mori is conceivable by data-mining techniques. We used the in silico cloning method to get the vacuolar-type $H^+-ATP$ synthetase (V-ATPase) c subunit (16 kDa proteolipid subunit) gene of B.mori and analysed with bioinformatics tools. The result was confirmed by RT-PCR and sequencing. The V-ATPase c subunit cDNA contains a 468 bp ORF. The ORF encoded a 155-residue protein that showed extensive homology with V-ATPase c subunits from other 15 species and contained four membrane-spanning helices. Tissue expression pattern analysis revealed that V-ATPase c expressed strongly in Malpighian tubules, not in fat body. This gene has been registered in GenBank under the accession number EU082222.