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.
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.
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.
Hypergolic propellants, which can ignite themselves without an ignition source, are difficult to handle due to their corrosiveness and toxicity. Therefore, it is necessary to develop green hypergolic propellants with little or no toxicity. In this study, basic research on green hypergolic ignition propellants was conducted. With 95% hydrogen peroxide as an oxidizer and CNU_HGFv1 as a fuel, ignition and combustion characteristics of propellants were evaluated through a drop test, an ignition test, and a combustion test. As a result of the drop test, the ignition delay time was 9.7 ms. It was 27 ms in the ignition test, which was fast enough to be used as a propellant. As a result of the combustion test, a combustion efficiency of 95.4~98.1% was achieved at about 11.7 bar. It was confirmed that fast and stable combustion was possible without hard start or combustion instability.
KSII Transactions on Internet and Information Systems (TIIS)
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v.17
no.8
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pp.2140-2156
/
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.
Proceedings of the Korea Information Processing Society Conference
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2008.11a
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pp.1332-1335
/
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.
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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v.12
no.4
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pp.263-271
/
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.
Yang Il-Seung;Yun Hyun-Do;Kim Do-Su;Khil Bae-Su;Han Seung-Gu
Journal of the Korea Concrete Institute
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v.17
no.5
s.89
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pp.769-774
/
2005
Fluosilicic acid(H2SiF6) is recovered as an aqueous solution which absorbs $SiF_4$ produced from the manufacturing of industrial-graded H3PO4 or HF. Generally, fluosilicates are the salts produced by the reaction of H2SiF6 and metal salts. Addition of fluosilicates to cement endows odd properties through unique chemical reaction with the fresh and hardened cement. This study was performed to know mechanical properties and watertightness using fluosilicates based composite made from fluosilicates and other compounds. Mix proportions for experiments were modulated at 0.45 of water to cement ratio and $0.0-2.0\%$ of adding ratio of fluosilicates based composite. Evaluation for mechanical properties of concrete was conducted to know fresh state of concrete, hardening state of concrete, and watertightness. Evaluation for watertightness of concrete was carried out permeability, absorption test and porosity analysis. In addition. Scanning Electron Microscopy(SEM) and Energy Dispersive X-Ray(EDX) used for investigating micro-structure and atomic component distributed in hardened concrete. It is ascertained that characteristics of mechanical properties and watertightness was more improved than non-added because of packing role of fluosilicates based composite and pozzolanic reaction of soluble $SiO_2$. Also, concrete added fluosilicates based composite had a tendency to delay setting time and only $0.5\%$ addition of fluosilicates based composite delayed 150 minutes compared with non-added.
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.
Seo, Chang-Woo;Yoon, Gil-Sang;Portugal, Sherlie;Hwang, In-Tae
Journal of the Institute of Electronics Engineers of Korea TC
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v.48
no.3
/
pp.6-12
/
2011
The fast growing of the number of users requires the development of reliable communication systems able to provide higher data rates. In order to meet those requirements, techniques such as Multiple Input Multiple Out (MIMO) and Orthogonal Frequency Division multiplexing (OFDM) have been developed in the recent years. In order to combine the benefits of both techniques, the research activity is currently focused on MIMO-OFDM systems. In addition, for a fast wireless channel environment, the data rate and reliability can be optimized by setting the modulation and coding adaptively according to the channel conditions; and using sub-carrier frequency, and power allocation techniques. Depending on how accurate the feedback-based system obtain the channel state information (CSI) and feed it back to the transmitter without delay, the overall system performance would be poor or optimal. In this paper, we propose a Signal to Noise Ratio (SNR) estimation algorithm where the preamble is known for both sides of the transciever. Through simulations made over several channel environments, we prove that our proposed SNR estimation algorithm is more accurate compared with the traditional SNR estimation.
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