• Title/Summary/Keyword: L2C signal

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A Case of Lethal Neonatal Type Carbamoyl Phosphate Synthetase 1 Deficiency with Novel Mutation of CPS1 (새로운 CPS1 유전자 돌연변이에 의한 신생아형 carbamoyl phosphate synthetase 1 결핍 1례)

  • Suh, Seung-hyun;Kim, Yoo-Mi;Byun, Shin Yun;Son, Seung Kook;Kim, Seong Heon;Kim, Hyung Tae;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.2
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    • pp.109-114
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    • 2016
  • Carbamoyl phosphate synthetase 1 (CPS1) deficiency is an autosomal recessive urea cycle disorder which causes hyperammonemia. CPS1 is the first enzyme step in the urea cycle and almost patients present their symptoms during neonatal period. We report a case of CPS1 deficiency in a boy who developed symptoms including lethargy and seizure at 3 days of age. The ammonia level was up to $2,325{\mu}mol/L$, sodium benzoate (250 mg/kg/d) and high calories of both dextrose and lipid was promptly administered. Central access by experienced pediatric surgeon and emergent continuous hemodialysis by pediatric nephrologist was performed within 3 hours and ammonia was less than $100{\mu}mol/L$ at 5 days of age. Currently, he has showed excellent response to treatments including scavenging drugs and a low-protein diet. Despite of diffuse increasing signal intensity on cerebral white matters and basal ganglia on brain MRI, his development and weight gain were good at the last follow-up at 11 months of age. Molecular assay of the CPS1 gene demonstrated that patient had compound heterozygous for c.1529del ($p.Gly510Alafs^*5$) in exon 14 and c.3142-1G>C (IVS25(-1)G>C) in intron 25 and exon 26 boundary. The splicing mutation was novel mutation and inherited from patient's mother. Here, we report a neonatal lethal type CPS1 deficiency patient having novel mutation.

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The Effect of Saccharin on the Gene Expression of NF-κB and Inflammatory Cytokines in LPS-Stimulated SW480 Colon Cancer Cells (옥수수수염 추출물이 SW480 Colon Cancer Cell에서 NF-κB와 염증성 사이토카인 발현에 미치는 영향)

  • Choi, Hyunji;Kim, Sunlim;Kang, Hyeonjung;Kim, Myunghwan;Kim, Wookyoung
    • Journal of the Korean Dietetic Association
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    • v.25 no.3
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    • pp.217-228
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    • 2019
  • There have been no published studies concerning the anti-inflammatory effects of corn silk on colon cancer cells. Thus, this study was conducted to investigate the effect of corn silk extract containing high levels of maysin on inflammation and its mechanism of action in colon cancer cells. SW 480 human colon cancer cells were treated with $1{\mu}g/mL$ of lipopolysaccharide (LPS) to induce inflammation, and next they were treated with different concentrations of corn silk extract (0, 5, 10 and $15{\mu}g/mL$). The concentrations of nitric oxide (NO) were determined. The mRNA expressions of inducible nitric oxide synthase (iNOS), cyclooxygenase-2 (COX-2), tumor necrosis factor ${\alpha}$ ($TNF-{\alpha}$), interleukin-1beta ($IL-1{\beta}$) and interleukin-6 (IL-6), were determined. Western blot analysis was performed to determine the protein expressions of nuclear factor-kappa B ($NF-{\kappa}B$) and mitogen-activated protein kinases, and the latter consists of extracellular signal-related kinase (ERK), c-jun NH2-terminal kinase (JNK) and p38 MAP kinase (p38). The concentration of NO and the mRNA expression of iNOS were significantly and dose-dependently decreased in the corn silk-treated groups (P<0.05). The mRNA expression of $TNF-{\alpha}$, $IL-1{\beta}$ and IL-6 were significantly increased in the LPS-treated group (P<0.05), but these expressions were significantly and dose-dependently decreased in the corn silk treated groups (P<0.05). The protein expressions of $NF-{\kappa}B$ (in a dose-dependent fashion), ERK (at 10 and $15{\mu}g/mL$), JNK (at $15{\mu}g/mL$) and p38 (at 10 and $15{\mu}g/mL$) were significantly decreased with corn silk treatments (P<0.05). In conclusion, corn silk extract containing high levels of maysin seems to inhibit the LPS-induced inflammatory responses in SW480 colon cancer cells via the $NF-{\kappa}B$ pathway.

The Significance of Acetylcholine Receptor Autoantibody Test (아세틸콜린 수용체 항체(Acetylcholine receptor autoantibody) 검사의 의의)

  • Yoo, Soh-Yeon;Lim, Soo-Yeon;Pack, Song-Ran;Seo, Mi-Hye;Moon, Hyung-Ho;You, Sun-Hee
    • The Korean Journal of Nuclear Medicine Technology
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    • v.15 no.1
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    • pp.113-116
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    • 2011
  • Purpose: Acetylcholine receptor antibodies cause acetylcholine receptor loss, which is responsible for failure of the neuromuscular junction in the acetylcholine receptor autoantibody. The disease characterized by muscle weakness and fatigue, myasthenia gravis(MG) occurs when the body inappropriately produces antibodies against acetylcholine receptors, and thus inhibits proper acetylcholine signal transmission. And this reason, the measurement of acetylcholine receptor antibodies can be of considerable value in disease diagnosis. Methods: From 2010. August to September, we tested orderd AchRAb 19 samples to get the results. 1. Pipette $5{\mu}{\ell}$ undiluted patient sera and kit control and add 125I AChR $50{\mu}{\ell}$ and incubate at R.T for 2 hours. 2. Pipette $50{\mu}{\ell}$ of anti-human IgG into each tube, and incubate at $2{\sim}8^{\circ}C$ for 2 hours. 3. Pipette $25{\mu}{\ell}$ precipitation enhancer into each tube and add 1mL washing solution into all tubes. 4. Centrifuge each tube for 20minutes at $2{\sim}8^{\circ}C$ at 1500g. 5. Aspirate or decant the supernatant. 6. Pipette 1 mL washing solution into all tubes and resuspend the pellet and repeat centrifugation. 7. Aspirate or decant the supernatant and count all tubes on a gamma counter. Results: Cut off value is 0.2 nmol/L and the results taken below 0.2 nmol/L are negative, the results above that identified as being positive values. We assayed the 19 patients samples and got 7 positive results. Of which, 6 patients were diagnosed as MG.(85.7%). Conclusions: Acetylcholine Receptor autoantibody test is intended for use by persons only for the quantitative determination of it in human serum. Even if measurement of the antibodies is not a routine test, it can be of considerable value in disease diagnosis.

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A STRAIN GAUGE ANALYSIS OF IMPLANT-SUPPORTED CANTILEVERED FIXED PROSTHESIS UNDER DISTAL STATIC LOAD

  • Sohn, Byoung-Sup;Heo, Seong-Joo;Chang, Ik-Tae;Koak, Jai-Young;Kim, Seong-Kyun
    • The Journal of Korean Academy of Prosthodontics
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    • v.45 no.6
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    • pp.717-723
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    • 2007
  • Statement of problem. Unreasonable distal cantilevered implant-supported prosthesis can mask functional problems of reconstruction temporarily, but it can cause serious strain and stress around its supported implant and surrounding alveolar bone. Purpose. The purpose of this study was to evaluate strain of implants supporting distal cantilevered fixed prosthesis with two different cantilevered length under distal cantilevered static load. Material and methods. A partially edentulous mandibular test model was fabricated with auto-polymerizing resin (POLYUROCK; Metalor technologies, Stuttgart, Swiss) and artificial denture teeth (Endura; Shofu inc., Kyoto, Japan). Two implants-supported 5-unit screw-retained cantilevered fixed prosthesis was made using standard methods with Type III gold alloy (Harmony C&B55; Ivoclar-vivadent, Liechtenstein, Germany) for superstructure and reinforced hard resin (Tescera; Ivoclar-vivadent, Liechtenstein, Germany) for occlusal material. Two strain gauges (KFG-1-120-C1-11L1M2R; KYOWA electronic instruments, Tokyo, Japan) were then attached to the mesial and the distal surface of each standard abutment with adhesive (M-bond 200; Tokuyama, Tokyo, Japan). Total four strain gauges were attached to test model and connected to dynamic signal conditioning strain amplifier (CTA1000; Curiotech inc., Paju, Korea). The stepped $20{\sim}100$ N in 25 N increments, cantilevered static load 8mm apart (Group I) or 16mm apart (Group II), were applied using digital push-pull gauge (Push-Pull Scale & Digital Force Gauge, Axis inc., Seoul, Korea). Each step was performed ten times and every strain signal was monitored and recorded. Results. In case of Group I, the strain values were surveyed by $80.7{\sim}353.8{\mu}m$ in Ch1, $7.5{\sim}47.9{\mu}m/m$ in Ch2, $45.7{\sim}278.6{\mu}m/m$ in Ch3 and $-212.2{\sim}718.7{\mu}m/m$ in Ch4 depending on increasing cantilevered static load. On the other hand, the strain values of Group II were surveyed by $149.9{\sim}612.8{\mu}m/m$ in Ch1, $26.0{\sim}168.5{\mu}m/m$ in Ch2, $114.3{\sim}632.3{\mu}m/m$ in Ch3, and $-323.2{\sim}-894.7{\mu}m/m$ in Ch4. Conclusion. A comparative statistical analysis using paired sample t-test about Group I Vs Group II under distal cantilevered load shows that there are statistical significant differences for all 4 channels (P<0.05).

Processed Panax ginseng, sun ginseng, inhibits the differentiation and proliferation of 3T3-L1 preadipocytes and fat accumulation in Caenorhabditis elegans

  • Lee, Hyejin;Kim, Jinhee;Park, Jun Yeon;Kang, Ki Sung;Park, Joeng Hill;Hwang, Gwi Seo
    • Journal of Ginseng Research
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    • v.41 no.3
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    • pp.257-267
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    • 2017
  • Background: Heat-processed ginseng, sun ginseng (SG), has been reported to have improved therapeutic properties compared with raw forms, such as increased antidiabetic, anti-inflammatory, and antihyperglycemic effects. The aim of this study was to investigate the antiobesity effects of SG through the suppression of cell differentiation and proliferation of mouse 3T3-L1 preadipocyte cells and the lipid accumulation in Caenorhabditis elegans. Methods: To investigate the effect of SG on adipocyte differentiation, levels of stained intracellular lipid droplets were quantified by measuring the oil red O signal in the lipid extracts of cells on differentiation Day 7. To study the effect of SG on fat accumulation in C. elegans, L4 stage worms were cultured on an Escherichia coli OP50 diet supplemented with $10{\mu}g/mL$ of SG, followed by Nile red staining. To determine the effect of SG on gene expression of lipid and glucose metabolism-regulation molecules, messenger RNA (mRNA) levels of genes were analyzed by real-time reverse transcription-polymerase chain reaction analysis. In addition, the phosphorylation of Akt was examined by Western blotting. Results: SG suppressed the differentiation of 3T3-L1 cells stimulated by a mixture of 3-isobutyl-1-methylxanthine, dexamethasone, and insulin (MDI), and inhibited the proliferation of adipocytes during differentiation. Treatment of C. elegans with SG showed reductions in lipid accumulation by Nile red staining, thus directly demonstrating an antiobesity effect for SG. Furthermore, SG treatment down-regulated mRNA and protein expression levels of peroxisome proliferator-activated receptor subtype ${\gamma}$ ($PPAR{\gamma}$) and CCAAT/enhancer-binding protein-alpha ($C/EBP{\alpha}$) and decreased the mRNA level of sterol regulatory element-binding protein 1c in MDI-treated adipocytes in a dose-dependent manner. In differentiated 3T3-L1 cells, mRNA expression levels of lipid metabolism-regulating factors, such as amplifying mouse fatty acid-binding protein 2, leptin, lipoprotein lipase, fatty acid transporter protein 1, fatty acid synthase, and 3-hydroxy-3-methylglutaryl coenzyme A reductase, were increased, whereas that of the lipolytic enzyme carnitine palmitoyltransferase-1 was decreased. Our data demonstrate that SG inversely regulated the expression of these genes in differentiated adipocytes. SG induced increases in the mRNA expression of glycolytic enzymes such as glucokinase and pyruvate kinase, and a decrease in the mRNA level of the glycogenic enzyme phosphoenol pyruvate carboxylase. In addition, mRNA levels of the glucose transporters GLUT1, GLUT4, and insulin receptor substrate-1 were elevated by MDI stimulation, whereas SG dose-dependently inhibited the expression of these genes in differentiated adipocytes. SG also inhibited the phosphorylation of Akt (Ser473) at an early phase of MDI stimulation. Intracellular nitric oxide (NO) production and endothelial nitric oxide synthase mRNA levels were markedly decreased by MDI stimulation and recovered by SG treatment of adipocytes. Conclusion: Our results suggest that SG effectively inhibits adipocyte proliferation and differentiation through the downregulation of $PPAR{\gamma}$ and $C/EBP{\alpha}$, by suppressing Akt (Ser473) phosphorylation and enhancing NO production. These results provide strong evidence to support the development of SG for antiobesity treatment.

Parallel Sub-filter Searching Structure and Parameter Decision Technique in Adaptive PN Code Acquisition Systems (적응형 확산 코드 동기획득 시스템에서의 병렬 부필터 탐색 구조와 파라미터 결정기법)

  • 한명수;류탁기;홍대식;강창언
    • The Journal of Korean Institute of Communications and Information Sciences
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    • v.28 no.7C
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    • pp.688-695
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    • 2003
  • In this paper, the parallel sub-filter searching structure to be adapted that divides a filter into several sub filters and the parameter decision technique to control adaptive parameters with power of received signal in the code acquisition system using adaptive filter are proposed. The numerical results for the system probabilities are derived that the code acquisition system using parallel sub-filter searching structure is statistically analyzed. Also, characteristic of the parameters in adaptive code acquisition system is analyzed by simulations and the parameter decision technique through SNRc estimation is explained. The system with parallel sub-filter searching structure outperforms by 1∼l.5 dB for 16 taps, 5∼6 dB for 64 taps. And the system with parameter decision technique works efficiently with the reasonable degree of degradation about 1 ∼2.5dB for 16 and 32 taps.

Anti-Inflammatory Effect of Chondrus nipponicus Yendo Ethanol Extract on Lipopolysaccharide-Induced Inflammatory Responses in RAW 264.7 Cells (LPS로 유도된 RAW 264.7 세포에 대한 가락진두발 에탄올 추출물의 항염증 효과)

  • Kim, Min-Ji;Bae, Nan-Yong;Kim, Koth-Bong-Woo-Ri;Park, Ji-Hye;Park, Sun-Hee;Jang, Mi-Ran;Ahn, Dong-Hyun
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.45 no.2
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    • pp.194-201
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    • 2016
  • The anti-inflammatory activity of ethanol extract from Chondrus nipponicus Yendo (CNYEE) was investigated by measuring production of a lipopolysaccharide-induced inflammatory response mediator. CNYEE had no cytotoxic effects on proliferation of macrophages compared to the control. CNYEE significantly inhibited (over 50%) NO production at $50{\mu}g/mL$, with inhibitory effects on expression levels of cytokines such as interleukin (IL)-6, tumor necrosis factor-${\alpha}$ (TNF-${\alpha}$), and IL-$1{\beta}$. In particular, IL-6 inhibitory activity of CNYEE was higher than 70% at $100{\mu}g/mL$. CNYEE also reduced protein expression of inducible nitric oxide synthase (iNOS), cyclooxygenase-2 (COX-2), and nuclear factor (NF)-${\kappa}B$ in a dose-dependent manner. CNYEE also significantly reduced phosphorylation of p38, extracellular signal-regulated kinase, and c-Jun N-terminal kinase. Therefore, these results suggest that CNYEE may have anti-inflammatory effects by modulating the NF-${\kappa}B$ and mitogen-activated protein kinases signaling pathways and may be used as an anti-inflammatory therapeutic material.

Fully Automated Liquid Culture System Compared with Lowenstein-Jensen Solid Medium for Rapid Recovery of Mycobacteria in Sputums (완전 자동화된 액체배양법과 기존의 고체배양법을 이용한 객담 내 mycobacterium의 신속검출에 대한 비교)

  • Park, Seung-Kyu;Kim, Seung-Chul;Kim, Deuk-Mi;Lee, Chang-Woon;Kim, Young;Cho, Sang-Nae
    • Tuberculosis and Respiratory Diseases
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    • v.53 no.6
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    • pp.635-643
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    • 2002
  • Background : The Aim of this study was to compare the recovery of mycobacteria from sputum samples of pulmonary tuberculosis patients using the MB/BacT rapid culture system(Organon Teknika, USA) with that obtained using Lowenstein-Jensen solid medium. Methods : The two culture systems were compared using sputum samples of 99 pulmonary tuberculosis patients. Culture media were incubated at $35-37^{\circ}C$ for six weeks in the MB/BacT system and for 12 weeks in Lowenstein-Jensen solid medium. Solid media were examined macroscopically once a week, and the MB/BacT system positive vials were unloaded from the machine as soon as possible after positive signal from the connected computer was detected Confirmation of growth for mycobacteria was done by Ziehl-Neelson stained smears. Isolates were identified to differentiate Mycobacterium tuberculosis from mycobacterium other than tuberculosis(MOTT) by phenotypic and molecular methods. Results : Of the sputum samples of the 99 patients, 58 samples were smear positive and 41 in negative smear. Mycobacteria were recovered from 67(67.7%) samples by using both culture systems. The yield with MB/BacT was higher than that with Lowenstein-Jensen [67(67.7%) vs. 52(52.5%), p<0.001]. Moreover, 15(15.2%) samples were positive only in the MB/BacT, whereas none of samples was positive only in Lowenstein-Jensen. In smear-positive and smear-negative samples, the recovery rate with MB/BacT was also higher than that with Lowenstein-Jensen [sputum-positive; 56/58(96.6%) vs. 46/58(79.3%), p=0.005, sputum-negative; 6/41(14.6%) vs. 5/41(12.2%), p<0.001]. The mean times to detection of Mycobacteria were 13.3 and 27.2 days with MB/BacT and Lowenstein-Jensen respectively(p<0.001). Conclusion : This results indicate that the the MB/BacT is more efficient and faster than Lowenstein-Jensen for the recovery of mycobacteria.

Speed Control of Three Phase Slotless PM BLDC Motor Using Single Sensor (Single Sensor를 이용한 3상 슬롯리스 PM BLDC 전동기의 속도제어)

  • Yoon Y. H.;Kim Y. C.;Lee S. S.;Won C. Y.;Choe Y. Y.
    • The Transactions of the Korean Institute of Power Electronics
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    • v.9 no.6
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    • pp.536-543
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    • 2004
  • Slotless Permanent Magnet Brushless DC Motor(PM BLDC) with the characteristics of high speed and power density has been more widely used In Industrial and factory machine. Generally, PM BLDC meter is necessary that the three Hall-lCs evenly be distributed around the stator circumference in case of the 3 phase motor. The Hall-ICs are set up in PM BLDC Motor to detect the main flux from the rotor. therefore the output signal from Hall-ICs is used to drive a power transistor to control the stator winding current. However, instead of using three Hall-ICs, if it used only one Hall-IC, we can estimate information of the others phase in sequence through a rotor This paper identified the characteristics and performance by using one Hall-IC with the 3-phase, 2-pole, 6-slot PM BLDC motor.

Performance Analysis of Electric Rail Car Office Using Computer Simulation (시뮬레이션을 이용한 전동차사무소 수행도 평가)

  • Lee, C.W.;Kim, W.Y;Kwon, Y.J;Kim, S.Y.;Yun, CH.;Oh, SJ;Jeon, T.B
    • Journal of Industrial Technology
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    • v.24 no.A
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    • pp.37-46
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    • 2004
  • A performance analysis for newly being considered electric rail car office has been made in this study. The major purpose is to examine a proposed design in terms of its capacity and the proposed number of travels (82-84) per day. For this study we first examined the overall system configuration with detailed operational processes of cleaning and inspection. We then developed a simulation model using ARENA and designed input data from 12 selected factors and their interaction effects. A simulation run for each treatment combination of $L_{16}(2^{15})$ orthogonal array was run and 20 batch means were obtained. Through careful analyses of the results obtained, we drew a diversity of suggestions including the best factor level combination. Our confirmation experiments at the optimal level combination further validate the possibility of 82 runs and the consistency in the results.

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