Search results for: model membrane

Authors: Dilina Janadith Nawarathne

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This article presents the initial development of a communication model (Model_isi) as the means of gathering, preserving and transferring indigenous knowledge in the field of knowledge management. The article first discusses the need for an appropriate complimentary model for indigenous knowledge management which differs from the existing methods and models. Then the paper suggests the newly developed model for indigenous knowledge management which generate as result of blending key aspects of different disciplines, which can be implemented as a complementary approach for the existing scientific method. The paper further presents the effectiveness of the developed method in reflecting upon a pilot demonstration carried out on selected indigenous communities of Sri Lanka.

Keywords: indigenous knowledge management, knowledge transferring, tacit knowledge, research model, asian centric philosophy

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Authors: Pingshan Chen, Zhiliang Dong

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In order to estimate the post-construction settlement more objectively, the power-polynomial model is proposed, which can reflect the trend of settlement development based on the observed settlement data. It was demonstrated by an actual case history of an embankment, and during the prediction. Compared with the other three prediction models, the power-polynomial model can estimate the post-construction settlement more accurately with more simple calculation.

Keywords: prediction, model, post-construction settlement, soft ground

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Authors: R. Shamsi, F. Sharifi

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In this paper, we obtain the projection of inefficient units in data envelopment analysis (DEA) in the case of stochastic inputs and outputs using the multi-objective programming (MOP) structure. In some problems, the inputs might be stochastic while the outputs are deterministic, and vice versa. In such cases, we propose a multi-objective DEA-R model because in some cases (e.g., when unnecessary and irrational weights by the BCC model reduce the efficiency score), an efficient decision-making unit (DMU) is introduced as inefficient by the BCC model, whereas the DMU is considered efficient by the DEA-R model. In some other cases, only the ratio of stochastic data may be available (e.g., the ratio of stochastic inputs to stochastic outputs). Thus, we provide a multi-objective DEA model without explicit outputs and prove that the input-oriented MOP DEA-R model in the invariable return to scale case can be replaced by the MOP-DEA model without explicit outputs in the variable return to scale and vice versa. Using the interactive methods for solving the proposed model yields a projection corresponding to the viewpoint of the DM and the analyst, which is nearer to reality and more practical. Finally, an application is provided.

Keywords: DEA-R, multi-objective programming, stochastic data, data envelopment analysis

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Authors: Katleho Daniel Makatjane, Diteboho Lawrence Xaba, Ntebogang Dinah Moroke

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The current study investigates the behaviour of time-varying parameters that are based on the score function of the predictive model density at time t. The mechanism to update the parameters over time is the scaled score of the likelihood function. The results revealed that there is high persistence of time-varying, as the location parameter is higher and the skewness parameter implied the departure of scale parameter from the normality with the unconditional parameter as 1.5. The results also revealed that there is a perseverance of the leptokurtic behaviour in stock returns which implies the returns are heavily tailed. Prior to model estimation, the White Neural Network test exposed that the stock price can be modelled by a GAS model. Finally, we proposed further researches specifically to model the existence of time-varying parameters with a more detailed model that encounters the heavy tail distribution of the series and computes the risk measure associated with the returns.

Keywords: generalized autoregressive score model, South Africa, stock returns, time-varying

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Authors: Abdur Rosyid, Mohamed El-Madany, Mohanad Alata

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Due to simplicity and low cost, rotordynamic system is often modeled by using lumped parameters. Recently, finite elements have been used to model rotordynamic system as it offers higher accuracy. However, it involves high degrees of freedom. In some applications such as control design, this requires higher cost. For this reason, various model reduction methods have been proposed. This work demonstrates the quality of model reduction of rotor-bearing-support system through substructuring. The quality of the model reduction is evaluated by comparing some first natural frequencies, modal damping ratio, critical speeds and response of both the full system and the reduced system. The simulation shows that the substructuring is proven adequate to reduce finite element rotor model in the frequency range of interest as long as the numbers and the locations of master nodes are determined appropriately. However, the reduction is less accurate in an unstable or nearly-unstable system.

Keywords: rotordynamic, finite element model, timoshenko beam, 3D solid elements, Guyan reduction method

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Authors: N. Naga Subrahmanyeswara Rao, Parag Arvind Deshpande

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Understanding nucleotide synthesis reaction of any organism is beneficial to know the growth of it as in Mycobacterium tuberculosis to design anti TB drug. One of the reactions of de novo pathway which takes place in all organisms was considered. The reaction takes places between phosphoribosyl pyrophosphate and orotate catalyzed by orotate phosphoribosyl transferase and divalent metal ion gives orotdine monophosphate, a nucleotide. All the reaction steps of three experimentally proposed mechanisms for this reaction were considered to develop kinetic rate expression. The model was validated using the data for four organisms. This model could successfully describe the kinetics for the reported data. The developed model can serve as a reliable model to describe the kinetics in new organisms without the need of mechanistic determination. So an organism-independent model was developed.

Keywords: mechanism, nucleotide, organism, tuberculosis

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Authors: C. Cohen, O. Levi

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This work introduces a generalization of the classical Tullock’s model of one-stage contests under complete information with multiple unlimited numbers of contestants. In classical Tullock’s model, the contest winner is not necessarily the highest bidder. Instead, the winner is determined according to a draw in which the winning probabilities are the relative contestants’ efforts. The Tullock modeling fits well political contests, in which the winner is not necessarily the highest effort contestant. This work presents a modified model which uses a simple non-discriminating rule, namely, a parameter to influence the total costs planned for an election, for example, the contest designer can control the contestants' efforts. The winner pays a fee, and the losers are reimbursed the same amount. Our proposed model includes a mechanism that controls the efforts exerted and balances competition, creating a tighter, less predictable and more interesting contest. Additionally, the proposed model follows the fairness criterion in the sense that it does not alter the contestants' probabilities of winning compared to the classic Tullock’s model. We provide an analytic solution for the contestant's optimal effort and expected reward.

Keywords: contests, Tullock's model, political elections, control expenses

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Authors: Deeksha Arora

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The study aims to explore the applicability of the widely used asset pricing models, namely, Capital Asset Pricing Model (CAPM) and the Fama-French Four Factor Model in the Indian equity market. The study will be based on the companies that form part of the Nifty Fifty Index for a period of five years: 2011 to 2016. The asset pricing model is examined by forming portfolios on the basis of three variables – market capitalization (size effect), book-to-market equity ratio (value effect) and profitability. The study provides a basis to test the presence of the Fama-French Four factor model in Indian stock market. This study may provide a basis for future research in the generalized asset pricing model comprising of multiple risk factors.

Keywords: book to market equity, Fama French four factor model, market capitalization, profitability, size effect, value effect

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Authors: Abdellahi Cheikh

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With the emergence of quantum computers with very powerful capabilities, the security of the exchange of shared keys between two interlocutors poses a big problem in terms of the rapid development of technologies such as computing power and computing speed. Therefore, the Diffie-Hellmann (DH) algorithm is more vulnerable than ever. No mechanism guarantees the security of the key exchange, so if an intermediary manages to intercept it, it is easy to intercept. In this regard, several studies have been conducted to improve the security of key exchange between two interlocutors, which has led to interesting results. The modification made on our model Diffie-Hellman-RSA-AES (DRA), which encrypts the information exchanged between two users using the three-encryption algorithms DH, RSA and AES, by using stenographic photos to hide the contents of the p, g and ClesAES values that are sent in an unencrypted state at the level of DRA model to calculate each user's public key. This work includes a comparative study between the DRA model and all existing solutions, as well as the modification made to this model, with an emphasis on the aspect of reliability in terms of security. This study presents a simulation to demonstrate the effectiveness of the modification made to the DRA model. The obtained results show that our model has a security advantage over the existing solution, so we made these changes to reinforce the security of the DRA model.

Keywords: Diffie-Hellmann, DRA, RSA, advanced encryption standard

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Authors: Mehrzad Abdi Khalife, Iraj Mahdavi

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This paper presents the agile model for project management process. For project management process, the Project Management Body of Knowledge (PMBOK) guideline has been selected as platform. Combination of computational science and artificial intelligent methodology has been added to the guideline to transfer the standard to agile project management process. The model is the combination of practical standard, computational science and artificial intelligent. In this model, we present communication model and protocols to keep process agile. Here, we illustrate the collaboration man and machine in project management area with artificial intelligent approach.

Keywords: artificial intelligent, conceptual model, man-machine collaboration, project management, standard

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Authors: Manoja Rajalakshmi Aravindakshana, Devleena Ghosha, Chittaranjan Mandala, K. V. Venkateshb, Jit Sarkarc, Partha Chakrabartic, Sujay K. Maity

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Oral Glucose Tolerance Test (OGTT) is the primary test used to diagnose type 2 diabetes mellitus (T2DM) in a clinical setting. Analysis of OGTT data using the Oral Minimal Model (OMM) along with the rate of appearance of ingested glucose (Ra) is performed to study differences in model parameters for control and T2DM groups. The differentiation of parameters of the model gives insight into the behaviour and physiology of T2DM. The model is also studied to find parameter differences among obese and non-obese T2DM subjects and the sensitive parameters were co-related to the known physiological findings. Sensitivity analysis is performed to understand changes in parameter values with model output and to support the findings, appropriate statistical tests are done. This seems to be the first preliminary application of the OMM with obesity as a distinguishing factor in understanding T2DM from estimated parameters of insulin-glucose model and relating the statistical differences in parameters to diabetes pathophysiology.

Keywords: oral minimal model, OGTT, obese and non-obese T2DM, mathematical modeling, parameter estimation

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Authors: Khaled Salah

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Model order reduction has been one of the most challenging topics in the past years. In this paper, a hybrid solution of genetic algorithm (GA) and simulated annealing algorithm (SA) are used to approximate high-order transfer functions (TFs) to lower-order TFs. In this approach, hybrid algorithm is applied to model order reduction putting in consideration improving accuracy and preserving the properties of the original model which are two important issues for improving the performance of simulation and computation and maintaining the behavior of the original complex models being reduced. Compared to conventional mathematical methods that have been used to obtain a reduced order model of high order complex models, our proposed method provides better results in terms of reducing run-time. Thus, the proposed technique could be used in electronic design automation (EDA) tools.

Keywords: genetic algorithm, simulated annealing, model reduction, transfer function

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Authors: Jessemyn Modiini, Timothy Lynar, Elena Sitnikova

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We present a novel enhanced compartmental model for malware spread analysis in cyber security. This paper applies cyber security data features to epidemiological compartmental models to model the infectious potential of malware. Compartmental models are most efficient for calculating the infectious potential of a disease. In this paper, we discuss and profile epidemiologically relevant data features from a Domain Name System (DNS) dataset. We then apply these features to epidemiological compartmental models to network traffic features. This paper demonstrates how epidemiological principles can be applied to the novel analysis of key cybersecurity behaviours and trends and provides insight into threat modelling above that of kill-chain analysis. In applying deterministic compartmental models to a cyber security use case, the authors analyse the deficiencies and provide an enhanced stochastic model for cyber epidemiology. This enhanced compartmental model (SUEICRN model) is contrasted with the traditional SEIR model to demonstrate its efficacy.

Keywords: cybersecurity, epidemiology, cyber epidemiology, malware

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Authors: N. V. Kuznetsov, O. A. Kuznetsova, G. A. Leonov, M. V. Yuldashev, R. V. Yuldashev

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Nonlinear analysis of the phase locked loop (PLL)-based circuits is a challenging task. Thus, the simulation is widely used for their study. In this work, we consider a mathematical model of the optical Costas loop and demonstrate the limitations of simulation approach related to the existence of so-called hidden oscillations in the phase space of the model.

Keywords: optical Costas loop, mathematical model, simulation, hidden oscillation

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Authors: Abdel-Mohdy, A. Abou-Okeil, S. El-Sabagh, S. M. El-Sawy

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Chitosan polyacrylic acid composite membranes were prepared by a bulk polymerization method in the presence of N, N'-methylene bisacrylamide (crosslinker) and ammonium persulphate as initiator. Membranes prepared from this copolymer in presence and absence of Ag nanoparticles were characterized by measuring mechanical and physical properties, water up-take and antibacterial properties. The results obtained indicated that the prepared membranes have antibacterial properties which increases with adding Ag nanoparticles.

Keywords: Ag nanoparticles , antimicrobial, Membrane, composites, mechanical properties, physical properties

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Authors: Julio Kauss, Miguel Cadillo, David Mauricio

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E-commerce is a business model that allows companies to optimize the processes of buying, selling, transferring goods and exchanging services through computer networks or the Internet. In Peru, the electronic commerce is used infrequently. This situation is due, in part to the fact that there is no model that allows companies to implement an e-commerce solution, which means that most SMEs do not have adequate knowledge to adapt to electronic commerce. In this work, a reference model is proposed for the implementation of an e-commerce solution in Peruvian SMEs in the retail sector. It consists of five phases: Business Analysis, Business Modeling, Implementation, Post Implementation and Results. The present model was validated in a SME of the Peruvian retail sector through the implementation of an electronic commerce platform, through which the company increased its sales through the delivery channel by 10% in the first month of deployment. This result showed that the model is easy to implement, is economical and agile. In addition, it allowed the company to increase its business offer, adapt to e-commerce and improve customer loyalty.

Keywords: e-commerce, retail, SMEs, reference model

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Authors: Do-Jin Jang, Sung-Ah Kim

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In designing a kinetic façade, it is hard for the designer to make digital models due to its complex geometry with motion. This paper aims to present a methodology of converting a point cloud of a physical model into a single digital model with a certain topology and motion. The method uses a Microsoft Kinect sensor, and color markers were defined and applied to three paper folding-inspired designs. Although the resulted digital model cannot represent the whole folding range of the physical model, the method supports the designer to conduct a performance-oriented design process with the rough physical model in the reduced folding range.

Keywords: design media, kinetic facades, tangible user interface, 3D scanning

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Authors: Yake Zhang, Peng Xu

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The development of building energy models (BEM) required for architectural design and analysis is a time-consuming and complex process, demanding a deep understanding and proficient use of simulation software. To streamline the generation of complex building energy models, this study proposes an automated method for generating building energy models using a large language model and the BEM library aimed at improving the efficiency of model generation. This method leverages a large language model to parse user-specified requirements for target building models, extracting key features such as building location, window-to-wall ratio, and thermal performance of the building envelope. The BEM library is utilized to retrieve energy models that match the target building’s characteristics, serving as reference information for the large language model to enhance the accuracy and relevance of the generated model, allowing for the creation of a building energy model that adapts to the user’s modeling requirements. This study enables the automatic creation of building energy models based on natural language inputs, reducing the professional expertise required for model development while significantly decreasing the time and complexity of manual configuration. In summary, this study provides an efficient and intelligent solution for building energy analysis and simulation, demonstrating the potential of a large language model in the field of building simulation and performance modeling.

Keywords: artificial intelligence, building energy modelling, building simulation, large language model

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Authors: Houcine Naim, Abdelatif Hassini, Noureddine Benabadji, Alex Van Den Bossche

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In this paper, two models to estimate the overall monthly average daily radiation on a horizontal surface were applied to the site of Oran (35.38 ° N, 0.37 °W). We present a comparison between the first one is a regression equation of the Angstrom type and the second model is developed by the present authors some modifications were suggested using as input parameters: the astronomical parameters as (latitude, longitude, and altitude) and meteorological parameters as (relative humidity). The comparisons are made using the mean bias error (MBE), root mean square error (RMSE), mean percentage error (MPE), and mean absolute bias error (MABE). This comparison shows that the second model is closer to the experimental values that the model of Angstrom.

Keywords: meteorology, global radiation, Angstrom model, Oran

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Authors: Nurulnadwan Aziz, Ariffin Abdul Mutalib, Siti Mahfuzah Sarif

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This paper reports an ongoing project regarding the development of Conceptual Design Model of Assistive Courseware for Low Vision (AC4LV) learners. Having developed the intended model, it has to be validated prior to producing it as guidance for the developers to develop an AC4LV. This study requires two phases of validation process which are through expert review and prototyping method. This paper presents a part of the validation process which is findings from experts review on Conceptual Design Model of AC4LV which has been carried out through a questionnaire. Results from 12 international and local experts from various respectable fields in Human-Computer Interaction (HCI) were discussed and justified. In a nutshell, reviewed Conceptual Design Model of AC4LV was formed. Future works of this study are to validate the reviewed model through prototyping method prior to testing it to the targeted users.

Keywords: assistive courseware, conceptual design model, expert review, low vision learners

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Authors: Ehsan Mehryaar

Abstract:

The rate of penetration is (RoP) one of the vital factors in the cost and time of tunnel boring projects; therefore, predicting it can lead to a substantial increase in the efficiency of the project. RoP is heavily dependent geological properties of the project site and TBM properties. In this study, 151-point data from Queen’s water tunnel is collected, which includes unconfined compression strength, peak slope index, angle with weak planes, and distance between planes of weaknesses. Since the size of the data is small, it was observed that it is imbalanced. To solve that problem synthetic minority oversampling technique is utilized. The model based on the model tree is proposed, where each leaf consists of a support vector machine model. Proposed model performance is then compared to existing empirical equations in the literature.

Keywords: Model tree, SMOTE, rate of penetration, TBM(tunnel boring machine), SVM

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Authors: Ghebremedhin Tsegay, Weldu Tesfagaber, Yuanmao Zhu, Xijun He, Wan Wang, Zhenjiang Zhang, Encheng Sun, Jinya Zhang, Yuntao Guan, Fang Li, Renqiang Liu, Zhigao Bu, Dongming Zhao*

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African swine fever (ASF) is a highly infectious viral disease of pigs, resulting in significant economic loss worldwide. As there is no approved vaccines and treatments, the control of ASF entirely depends on early diagnosis and culling of infected pigs. Thus, highly specific and sensitive diagnostic assays are required for accurate and early diagnosis of ASF virus (ASFV). Currently, only a few recombinant proteins have been tested and validated for use as reagents in ASF diagnostic assays. The most promising ones for ASFV antibody detection were p72, p30, p54, and pp62. So far, three ELISA kits based on these recombinant proteins have been commercialized. Due to the complex nature of the virus and variety forms of the disease, robust serodiagnostic assays are still required. ASFV p22 protein, encoded by KP177R gene, is located in the inner membrane of viral particle and appeared transiently in the plasma membrane early after virus infection. The p22 protein interacts with numerous cellular proteins, involved in processes of phagocytosis and endocytosis through different cellular pathways. However, p22 does not seem to be involved in virus replication or swine pathogenicity. In this study, E.coli expressed recombinant p22 protein was used to generate a monoclonal antibody (mAb), and its potential use for the development of blocking ELISA (bELISA) was evaluated. A total of 806 pig serum samples were tested to evaluate the bELISA. Acording the ROC (Reciever operating chracteristic) analysis, 100% sensitivity and 98.10% of specificity was recorded when the PI cut-off value was set at 47%. The novel assay was able to detect the antibodies as early as 9 days post infection. Finaly, a highly sensitive, specific and rapid novel p22-mAb based bELISA assay was developed, and optimized for detection of antibodies against genotype I and II ASFVs. It is a promising candidate for an early and acurate detection of the antibodies and is highly expected to have a valuable role in the containment and prevention of ASF.

Keywords: ASFV, blocking ELISA, diagnosis, monoclonal antibodies, sensitivity, specificity

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Authors: Sina Saadati, Mohammadreza Razzazi

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In this article, we have tried to present an agent-based model of human muscle. A suitable model of muscle is necessary for the analysis of mankind's movements. It can be used by clinical researchers who study the influence of motion sicknesses, like Parkinson's disease. It is also useful in the development of a prosthesis that receives the electromyography signals and generates force as a reaction. Since we have focused on computational efficiency in this research, the model can compute the calculations very fast. As far as it concerns prostheses, the model can be known as a charge-efficient method. In this paper, we are about to illustrate an agent-based model. Then, we will use it to simulate the human gait cycle. This method can also be done reversely in the analysis of gait in motion sicknesses.

Keywords: agent-based modeling and simulation, human muscle, gait cycle, motion sickness

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Authors: Mustafa Yılmaz, Ahmet Akar, Nesrin Köken, Nilgün Kızılcan

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Commercially available poly(acrylonitrile-co-vinyl acetate) P(AN-VA) or poly(acrylonitrile-co-methyl acrylate) P(AN-MA) are not satisfactory to meet the demand in flame and fire-resistance. In this work, vinylphosphonic acid is used during polymerization of acrylonitrile, vinyl acetate, methacrylic acid to produce fire-retardant polymers. These phosphorus containing polymers are successfully spun in the form of nanofibers. Properties such as water absorption of polymers are also determined and compared with commercial polymers.

Keywords: flame retardant, nanofiber, polyacrylonitrile, phosphorous compound, membrane

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Authors: Wanhyun Cho, Soonja Kang, Sanggoon Kim, Soonyoung Park

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We present probabilistic multinomial Dirichlet classification model for multidimensional data and Gaussian process priors. Here, we have considered an efficient computational method that can be used to obtain the approximate posteriors for latent variables and parameters needed to define the multiclass Gaussian process classification model. We first investigated the process of inducing a posterior distribution for various parameters and latent function by using the variational Bayesian approximations and important sampling method, and next we derived a predictive distribution of latent function needed to classify new samples. The proposed model is applied to classify the synthetic multivariate dataset in order to verify the performance of our model. Experiment result shows that our model is more accurate than the other approximation methods.

Keywords: multinomial dirichlet classification model, Gaussian process priors, variational Bayesian approximation, importance sampling, approximate posterior distribution, marginal likelihood evidence

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Authors: Mahacine Amrani

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This paper aims at reviewing some Moroccan industrial applications of wavelet especially in the dynamic identification of a process model using Haar wavelet mother response. Two recent Moroccan study cases are described using dynamic data originated by a distillation column and an industrial polyethylene process plant. The purpose of the wavelet scheme is to build on-line dynamic models. In both case studies, a comparison is carried out between the Haar wavelet mother response model and a linear difference equation model. Finally it concludes, on the base of the comparison of the process performances and the best responses, which may be useful to create an estimated on-line internal model control and its application towards model-predictive controllers (MPC). All calculations were implemented using AutoSignal Software.

Keywords: process performance, model, wavelets, Haar, Moroccan

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Authors: Okike Benjamin, Garba E. J. D.

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The researcher has developed a new encryption technique known as Merged Irregular Transposition Cipher. In this cipher method of encryption, a message to be encrypted is split into parts and each part encrypted separately. Before the encrypted message is transmitted to the recipient(s), the positions of the split in the encrypted messages could be swapped to ensure more security. This work seeks to develop a model by considering the split number, S and the average number of characters per split, L as the message under consideration is split from 2 through 10. Again, after developing the model, the error level in the model would be determined.

Keywords: merged irregular transposition, error level, model estimation, message splitting

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Authors: Mohanaad Talal Shakir

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This paper tries to propose educational design by using multimedia technology for Engineering of computer Technology, Alma'ref University College in Iraq. This paper evaluates the acceptance, cognition, and interactiveness of the proposed model by students by using the statistical relationship to determine the stage of the model. Objectives of proposed education design are to develop a user-friendly software for education purposes using multimedia technology and to develop animation for 3D model to simulate assembling and disassembling process of high-speed flow.

Keywords: CAL, multimedia, shock tunnel, interactivity, engineering education

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Authors: Zhidong Zhang, Yingchen Yang

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In this study, a diagnostic assessment model for Mastery Engineering Learning was established based on a group of undergraduate students who studied in an engineering course. A diagnostic assessment model can examine both students' learning process and report achievement results. One very unique characteristic is that the diagnostic assessment model can recognize the errors and anything blocking students in their learning processes. The feedback is provided to help students to know how to solve the learning problems with alternative strategies and help the instructor to find alternative pedagogical strategies in the instructional designs. Dynamics is a core course in which is a common course being shared by several engineering programs. This course is a very challenging for engineering students to solve the problems. Thus knowledge acquisition and problem-solving skills are crucial for student success. Therefore, developing an effective and valid assessment model for student learning are of great importance. Diagnostic assessment is such a model which can provide effective feedback for both students and instructor in the mastery of engineering learning.

Keywords: diagnostic assessment, mastery learning, engineering, bayesian network model, learning processes

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Authors: Ozlem Oral, Emre Taskin, Aysel Yuce, Serap Dokmeci, Devrim Gozuacik

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Autophagy is an evolutionary conserved lysosome-dependent catabolic pathway, responsible for the degradation of long-lived proteins, abnormal aggregates and damaged organelles which cannot be degraded by the ubiquitin-proteasome system. Lysosomes degrade the substrates through the activity of lysosomal hydrolases and lysosomal membrane-bound proteins. Mutations in the coding region of these proteins cause malfunctional lysosomes, which contributes to the pathogenesis of lysosomal storage diseases. Gaucher disease is a lysosomal storage disease resulting from the mutation of a lysosomal membrane-associated glycoprotein called glucocerebrosidase and its cofactor saposin C. The disease leads to intracellular accumulation of glucosylceramide and other glycolipids. Because of the essential role of lysosomes in autophagic degradation, Gaucher disease may directly be linked to this pathway. In this study, we investigated the expression of autophagy and/or lysosome-related genes and proteins in fibroblast cells isolated from patients with different mutations. We carried out confocal microscopy analysis and examined autophagic flux by utilizing the differential pH sensitivities of RFP and GFP in mRFP-GFP-LC3 probe. We also evaluated lysosomal pH by active lysosome staining and lysosomal enzyme activity. Beside lysosomes, we also performed proteasomal activity and cell death analysis in patient samples. Our data showed significant attenuation in the expression of key autophagy-related genes and accumulation of their proteins in mutant cells. We found decreased the ability of autophagosomes to fuse with lysosomes, associated with elevated lysosomal pH and reduced lysosomal enzyme activity. Proteasomal degradation and cell death analysis showed reduced proteolytic activity of the proteasome, which consequently leads to increased susceptibility to cell death. Our data indicate that the major degradation pathways are affected by multifunctional lysosomes in mutant patient cells and may underlie in the mechanism of clinical severity of Gaucher patients. (This project is supported by TUBITAK-3501-National Young Researchers Career Development Program, Project No: 112T130).

Keywords: autophagy, Gaucher's disease, glucocerebrosidase, mutant fibroblasts

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