• 제목/요약/키워드: State Delay

검색결과 951건 처리시간 0.029초

Homozygous Missense Epithelial Cell Adhesion Molecule Variant in a Patient with Congenital Tufting Enteropathy and Literature Review

  • Guvenoglu, Merve;Simsek-Kiper, Pelin Ozlem;Kosukcu, Can;Taskiran, Ekim Z.;Saltik-Temizel, Inci Nur;Gucer, Safak;Utine, Eda;Boduroglu, Koray
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제25권6호
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    • pp.441-452
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    • 2022
  • Congenital diarrheal disorders (CDDs) with genetic etiology are uncommon hereditary intestinal diseases characterized by chronic, life-threatening, intractable watery diarrhea that starts in infancy. CDDs can be mechanistically divided into osmotic and secretory diarrhea. Congenital tufting enteropathy (CTE), also known as intestinal epithelial dysplasia, is a type of secretory CDD. CTE is a rare autosomal recessive enteropathy that presents with intractable neonatal-onset diarrhea, intestinal failure, severe malnutrition, and parenteral nutrition dependence. Villous atrophy of the intestinal epithelium, crypt hyperplasia, and irregularity of surface enterocytes are the specific pathological findings of CTE. The small intestine and occasionally the colonic mucosa include focal epithelial tufts. In 2008, Sivagnanam et al. discovered that mutations in the epithelial cell adhesion molecule (EpCAM, MIM# 185535) were the genetic cause of CTE (MIM# 613217). More than a hundred mutations have been reported to date. Furthermore, mutations in the serine peptidase inhibitor Kunitz type 2 (SPINT2, MIM# 605124) have been linked to syndromic CTE. In this study, we report the case of a 17-month-old male infant with congenital diarrhea. Despite extensive etiological workup, no etiology could be established before admission to our center. The patient died 15 hours after being admitted to our center in a metabolically decompensated state, probably due to a delay in admission and diagnosis. Molecular autopsy with exome sequencing revealed a previously reported homozygous missense variant, c.757G>A, in EpCAM, which was confirmed by histopathological examination.

RNA Binding Protein Rbms1 Enables Neuronal Differentiation and Radial Migration during Neocortical Development by Binding and Stabilizing the RNA Message for Efr3a

  • Habib, Khadija;Bishayee, Kausik;Kang, Jieun;Sadra, Ali;Huh, Sung-Oh
    • Molecules and Cells
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    • 제45권8호
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    • pp.588-602
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    • 2022
  • Various RNA-binding proteins (RBPs) are key components in RNA metabolism and contribute to several neurodevelopmental disorders. To date, only a few of such RBPs have been characterized for their roles in neocortex development. Here, we show that the RBP, Rbms1, is required for radial migration, polarization and differentiation of neuronal progenitors to neurons in the neocortex development. Rbms1 expression is highest in the early development in the developing cortex, with its expression gradually diminishing from embryonic day 13.5 (E13.5) to postnatal day 0 (P0). From in utero electroporation (IUE) experiments when Rbms1 levels are knocked down in neuronal progenitors, their transition from multipolar to bipolar state is delayed and this is accompanied by a delay in radial migration of these cells. Reduced Rbms1 levels in vivo also reduces differentiation as evidenced by a decrease in levels of several differentiation markers, meanwhile having no significant effects on proliferation and cell cycle rates of these cells. As an RNA binding protein, we profiled the RNA binders of Rbms1 by a cross-linked-RIP sequencing assay, followed by quantitative real-time polymerase chain reaction verification and showed that Rbms1 binds and stabilizes the mRNA for Efr3a, a signaling adapter protein. We also demonstrate that ectopic Efr3a can recover the cells from the migration defects due to loss of Rbms1, both in vivo and in vitro migration assays with cultured cells. These imply that one of the functions of Rbms1 involves the stabilization of Efr3a RNA message, required for migration and maturation of neuronal progenitors in radial migration in the developing neocortex.

Effects of Pahs and Pcbs and Their Toxic Metabolites on Inhibition of Gjic and Cell Proliferation in Rat Liver Epithelial Wb-F344 Cells

  • Miroslav, Machala;Jan, Vondracek;Katerina, Chramostova;Lenka, Sindlerova;Pavel, Krcmar;Martina, Pliskova;Katerina, Pencikova;Brad, Upham
    • 한국환경성돌연변이발암원학회지
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    • 제23권2호
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    • pp.56-62
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    • 2003
  • The liver progenitor cells could form a potential target cell population fore both tumor-initiating and -promoting chemicals. Induction of drug-metabolizing and antioxidant enzymes, including AhR-dependent CYP1A1, NQO-1 and AKR1C9, was detected in the rat liver epithelial WB-F344 "stem-like" cells. Additionally, WB-F344 cells express a functional, wild-type form of p53 protein, a biomarker of genotoxic events, and connexin 43, a basic structural unit of gap junctions forming an important type of intercellular communication. In this cellular model, two complementary assays have been established for detection of the modes of action associated with tumor promotion: inhibition of gap junctional intercellular communication (GJIC) and proliferative activity in confluent cells. We found that the PAHs and PCBs, which are AhR agonists, released WB-F344 cells from contact inhibition, increasing both DNA synthesis and cell numbers. Genotoxic effects of some PAHs that lead to apoptosis and cell cycle delay might interfere with the proliferative activity of PAHs. Contrary to that, the nongenotoxic low-molecular-weight PAHs and non-dioxin-like PCB congeners, abundant in the environment, did not significantly affect cell cycle and cell proliferation; however both groups of compounds inhibited GJIC in WB-F344 cells. The release from contact inhibiton by a mechanism that possibly involves the AhR activation, inhibition of GJIC and genotoxic events induced by environmental contaminants are three important modes of action that could play an important role in carcinogenic effects of toxic compounds. The relative potencies to inhibit GJIC, to induce AhR-mediated activity, and to release cells from contact inhibition were determined for a large series of PAHs and PCBs and their metabolites. In vitro bioassays based on detection of events on cellular level (deregulation of GJIC and/or proliferation) or determination of receptor-mediated activities in both ?$stem-like^{\circ}{\times}$ and hepatocyte-like liver cellular models are valuable tools for detection of modes of action of polyaromatic hydrocarbons. They may serve, together with concentration data, as a first step in their risk assessment.

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친환경 접촉점화 추진제 점화 성능 평가 (Evaluation of Ignition Performance of Green Hypergolic Propellant)

  • 김선진;신민규;차정열;고영성
    • 항공우주시스템공학회지
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    • 제17권1호
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    • pp.51-58
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    • 2023
  • 별도의 점화원 없이 스스로 점화가 가능한 접촉점화 추진제는 부식성과 독성으로 인하여 취급의 어려움이 있다. 따라서 독성이 적거나 없는 친환경 접촉점화 추진제의 개발이 필요하며, 본 연구에는 친환경 접촉점화 추진제의 기초 연구를 수행하였다. 산화제로 95%의 과산화수소, 연료로 CNU_HGFv1를 사용하였으며 액적 낙하 시험, 점화 시험 및 연소 시험을 통하여 추진제의 점화 및 연소 특성을 확인하였다. 액적 낙하 시험 결과 점화지연 시간은 9.7ms 이며, 점화시험에서는 약 27ms로 추진제로 사용하기에 충분히 빠른 것을 확인하였다. 연소시험 결과 약 11.7bar에서 연소 효율 95.4~98.1%를 달성하였으며, 하드스타트 및 연소 불안정 없이 빠르고 안정적인 연소가 가능함을 확인하였다.

Energy Efficient Cluster Head Selection and Routing Algorithm using Hybrid Firefly Glow-Worm Swarm Optimization in WSN

  • Bharathiraja S;Selvamuthukumaran S;Balaji V
    • KSII Transactions on Internet and Information Systems (TIIS)
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    • 제17권8호
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    • pp.2140-2156
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    • 2023
  • The Wireless Sensor Network (WSN), is constructed out of teeny-tiny sensor nodes that are very low-cost, have a low impact on the environment in terms of the amount of power they consume, and are able to successfully transmit data to the base station. The primary challenges that are presented by WSN are those that are posed by the distance between nodes, the amount of energy that is consumed, and the delay in time. The sensor node's source of power supply is a battery, and this particular battery is not capable of being recharged. In this scenario, the amount of energy that is consumed rises in direct proportion to the distance that separates the nodes. Here, we present a Hybrid Firefly Glow-Worm Swarm Optimization (HF-GSO) guided routing strategy for preserving WSNs' low power footprint. An efficient fitness function based on firefly optimization is used to select the Cluster Head (CH) in this procedure. It aids in minimising power consumption and the occurrence of dead sensor nodes. After a cluster head (CH) has been chosen, the Glow-Worm Swarm Optimization (GSO) algorithm is used to figure out the best path for sending data to the sink node. Power consumption, throughput, packet delivery ratio, and network lifetime are just some of the metrics measured and compared between the proposed method and methods that are conceptually similar to those already in use. Simulation results showed that the proposed method significantly reduced energy consumption compared to the state-of-the-art methods, while simultaneously increasing the number of functioning sensor nodes by 2.4%. Proposed method produces superior outcomes compared to alternative optimization-based methods.

차별적이니 드랍-확률을 갖는 동적-VQSDDP를 이용한 상대적 손실차별화의 달성 (Achieving Relative Loss Differentiation using D-VQSDDP with Differential Drop Probability)

  • 조경래;구자환;정진욱
    • 한국정보처리학회:학술대회논문집
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    • 한국정보처리학회 2008년도 추계학술발표대회
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    • pp.1332-1335
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    • 2008
  • In order to various service types of real time and non-real time traffic with varying requirements are transmitted over the IEEE 802.16 standard is expected to provide quality of service(QoS) researchers have explored to provide a queue management scheme with differentiated loss guarantees for the future Internet. The sides of a packet drop rate, an each class to differential drop probability on achieving a low delay and high traffic intensity. Improved a queue management scheme to be enhanced to offer a drop probability is desired necessarily. This paper considers multiple random early detection with differential drop probability which is a slightly modified version of the Multiple-RED(Random Early Detection) model, to get the performance of the best suited, we analyzes its main control parameters (maxth, minth, maxp) for achieving the proportional loss differentiation (PLD) model, and gives their setting guidance from the analytic approach. we propose Dynamic-multiple queue management scheme based on differential drop probability, called Dynamic-VQSDDP(Variable Queue State Differential Drop Probability)T, is proposed to overcome M-RED's shortcoming as well as supports static maxp parameter setting values for relative and each class proportional loss differentiation. M-RED is static according to the situation of the network traffic, Network environment is very dynamic situation. Therefore maxp parameter values needs to modify too to the constantly and dynamic. The verification of the guidance is shown with figuring out loss probability using a proposed algorithm under dynamic offered load and is also selection problem of optimal values of parameters for high traffic intensity and show that Dynamic-VQSDDP has the better performance in terms of packet drop rate. We also demonstrated using an ns-2 network simulation.

Testing and evaluation of the corrosion behavior of Aluminum/Alumina bulk composites fabricated via combined stir casting and APB process

  • Abdalkareem Jasim;Ghassan Fadhil Smaisim;Abduladheem Turki Jalil;Surendar Aravindhan;Abdullah Hasan Jabbar;Shaymaa Abed Hussein;Muneam Hussein Ali;Muataz S. Alhassan;Yasser Fakri Mustafa
    • Advances in materials Research
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    • 제12권4호
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    • pp.263-271
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    • 2023
  • In this study, AA1060/Alumina composites were fabricated by combined stir casting and accumulative press bonding (APB). The APB process was repeated up to six press bonding steps at 300Ċ. As the novelty, potential dynamic polarization in 3.5Wt% NaCl solution was used to study the corrosion properties of these composites. The corrosion behavior of these samples was compared and studied with that of the annealed aluminum alloy 1060 and versus the number of APB steps. So, as a result of enhancing influence on the number of APB process, this experimental investigation showed a significant enhancement in the main electrochemical parameters and the inert character of the Alumina particles. Together with Reducing the active zones of the material surfaces could delay the corrosion process. Also, at higher number of steps, the corrosion resistance of composites improved. The sample produced after six number of steps had a low corrosion density in comparison with high corrosion density of annealed specimens. Also, the scanning electron microscopy (SEM), was used to study the corrosion surface of samples.

규불화염계 복합 조성물을 혼입한 콘크리트의 공학적 특성에 관한 실험적 연구 (Experimental Study on Engineering Properties of Concrete Using Fluosilicates Based Composite)

  • 양일승;윤현도;김도수;길배수;한승구
    • 콘크리트학회논문집
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    • 제17권5호
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    • pp.769-774
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    • 2005
  • 최근 무기 불소계 공정 부산물의 발생에 따른 환경 오염 문제 해결과 재활용을 위한 노력이 중요한 현안으로 대두되고 있다. 따라서 세계적으로 이러한 목적을 달성하기 위하여 각종 재활용 기술 및 제조 시설 등이 개발되고 있으며, 현재에도 끊임없는 기술 개발의 노력이 시도되고 있다. 이러한 추세에 따라 국내에서도 1990년대 후반부터 인산$(H_3PO_4)$ 및 불산(HF)을 제조하는 공정 중에 액상형태의 부산물로 회수되는 불화규산$(H_2SiF_6)$을 활용하여 안정한 액상형태로 제조되는 규불화염계 화합물이 콘크리트의 강도를 증진시키는 동시에 수밀성 증진 및 경화 후 물성에 긍정적인 영향을 주며, 향후 건설용 혼화재료로써의 새로운 기능성이 제기되고 있다. 본 연구에서는 인산 제조 공정 중에 공정부산물로 회수되는 불화규산$(H_2SiF_6)$을 활용하여 제조된 규불 화염계 복합 조성물을 첨가한 콘크리트의 공학적 특성을 파악하고자 콘크리트의 굳지 않은 특성(유동성, 공기량, 블리딩, 응결시간), 콘크리트의 경화 특성(압축강도, 길이 변화) 및 콘크리트 수밀성에 대하여 검토를 행하였다. 그 결과, 규불화염계 복합 조성물의 첨가하면 시멘트 수화 과정 중에 생성되는 난용성 금속불화물의 충전작용 및 가용성 실리카의 포졸란반응의 복합적 효과에 의해 압축강도가 향상되고 투수율 및 공극률이 크게 감소되어 콘크리트 경화체의 수밀성이 증진되는 것으로 확인되었다.

흰쥐 Tail Flick Reflex의 신경생리학적 기전 (Electrophysiologic Mechanism of Tail Flick Reflex in Rats)

  • 서상아;김전
    • The Korean Journal of Physiology
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    • 제23권1호
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    • pp.139-149
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    • 1989
  • Although tail flick reflex (TFR) in rats has been used as a classic model of the nociceptive test to evaluate the action of analgesics, there have been few studies on the origin of the latent period of TFR. Present study was performed to elucidate the mechanism of increase in latency of TFR by morphine in anesthetized rats. Tail skin and dorsolateral tail nerve were stimulated electrically and EMG activities were recorded from abductor caudae dorsalis muscle participating in tail flick reflex. In the case of noxious radiant heat stimulation to tail, the tail flick tension was recorded before and after administration of morphine. Then changes in latency and conduction velocity of peripheral nerve were evaluated. The results obtained were as follows: 1) The latencies of TFR evoked by the electrical stimulation of tail skin and dorsolateral tail nerve were all within 40 ms and were elongated by several milliseconds from control after the administration of morphine. Peripheral conduction velocities of tail flick afferent nerve were within the range of 10-25 m/s. 2) The conduction velocity of peripheral nerve was significantly reduced after morphine administration, therefore the afferent time (utilization time+conduction time to spinal cord) was significantly increased. But the time for central delay and efferent time was not affected by morphine. 3) The conduction velocity under room temperature $(20-25^{\circ}C)$ was significantly reduced after morphine while that under vasodilation state $(40{\sim}42^{\circ}C)$ increased, 30 min and 45 min after morphine. The conduction velocity under vasodilation state without treatment of morphine increased continuously 4) The latency in tension response of TFR evoked by electrical stimulation was elongated by several milliseconds from control while the latency evoked by noxious radiant heat was elongated by several seconds compared with that of control. From the above results, it could be concluded that: 1) the increased latency of TFR evoked by electrical stimulation of the tail after morphine administration was due to the reducton in conduction velocity of peripheral nerve, which was the secondry effect of morphine on the peripheral vasomotion and 2) increased latency of TFR evoked by noxious radiant heat was also due to the same effect of morphine and the increase in cutaneous insulation to the noxious heat.

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프리앰블을 이용한 새로운 SNR 추정 알고리즘 제안 및 성능 분석 (New SNR Estimation Algorithm using Preamble and Performance Analysis)

  • 서창우;윤길상;셰를리 포루투갈;황인태
    • 대한전자공학회논문지TC
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    • 제48권3호
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    • pp.6-12
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    • 2011
  • 이동통신이 발전해 감으로써 사용자들은 점점 더 높은 데이터율과 신뢰성이 높은 통신 시스템을 요구하고 있다. 최근에는 이러한 사용자 요구에 부합하기 위해 MIMO(Multiple Input Multiple Out)와 OFDM(Orthogonal Frequency Division multiplexing)의 장점을 모두 활용하는 MIMO-OFDM에 기반을 둔 시스템 개발이 활발히 이루어지고 있다. 또한, 빠른 무선 채널 환경에 대응하여 높은 전송률 및 양질의 데이터를 만족하기 위해서 채널 상태에 따라서 적응적으로 변조, 코딩, 부 반송 파수 및 파워 할당을 달리하는 시스템 등이 결합되고 있다. 이러한 피드백 기반 시스템은 얼마나 정확히 채널에 대한 상태 정보(CSI : Channel State Information)를 지연 없이 송신기에 전달하느냐에 따라서 시스템 전체 성능이 향상 될 수 있고 저하될 수 있다. 본 논문에서는 송 수신단에서 서로 알고 있는 프리앰블(Preamble)을 이용하여 채널 추정과정 없이 정확한 SNR (신호 대 잡음비: Signal to Noise Ratio) 추정이 가능한 알고리즘을 제안한다. 여러 채널 환경에서 수행한 모의 실험결과, 제안 알고리즘은 기존의 프리앰블 기반 SNR 추정 알고리즘들 보다 가장 정확하게 SNR을 추정하는 것을 확인할 수 있었다.