Miracle-dz/UniFAR-FTUs huggingface dataset:
Genotype: A Crucial but Not Unique Factor Affecting the Clinical Phenotypes in Fabry Disease

Numerous α-galactosidase A (α-gal A) gene (GLA) mutations have been identified in Fabry disease (FD), but studies on genotype-phenotype correlation are limited. This study evaluated the features of GLA gene mutations and genotype-phenotype relationship in Chinese FD patients. Gene sequencing results, demographic information, clinical history, and laboratory findings were collected from 73 Chinese FD patients. Totally 47 mutations were identified, including 23 novel mutations which might be pathogenic. For male patients, those with frameshift and nonsense mutations presented the classical FD, whereas those with missense mutations presented both of classical and atypical phenotypes. Interestingly, two male patients with missense mutation p.R356G from two unrelated families, and two with p.R301Q from one family presented different phenotypes. A statistically significant association was found between the levels of α-gal A enzyme activity and ocular changes in males, though no significant association was found between residual enzyme activity level and genotype or clinical phenotypes. For female patients, six out of seven with frameshift mutations and one out of nine with missense mutation presented the classical FD, and α-gal A activity in those patients was found to be significantly lower than that of patients with atypical phenotypes (13.73 vs. 46.32 nmol/ml/h/mg). Our findings suggest that the α-gal A activity might be associated with the clinical severity in female patients with FD. But no obvious associations between activity level of α-gal A and genotype or clinical phenotypes were found for male patients.
GaLactic and Extragalactic All-sky Murchison Widefield Array (GLEAM) survey - I. A low-frequency extragalactic catalogue

Using the Murchison Widefield Array (MWA), the low-frequency Square Kilometre Array precursor located in Western Australia, we have completed the GaLactic and Extragalactic All-sky MWA (GLEAM) survey and present the resulting extragalactic catalogue, utilizing the first year of observations. The catalogue covers 24 831 square degrees, over declinations south of +30° and Galactic latitudes outside 10° of the Galactic plane, excluding some areas such as the Magellanic Clouds. It contains 307 455 radio sources with 20 separate flux density measurements across 72–231 MHz, selected from a time- and frequency-integrated image centred at 200 MHz, with a resolution of ≈ 2 arcmin. Over the catalogued region, we estimate that the catalogue is 90 per cent complete at 170 mJy and 50 per cent complete at 55 mJy and large areas are complete at even lower flux density levels. Its reliability is 99.97 per cent above the detection threshold of 5σ, which itself is typically 50 mJy. These observations constitute the widest fractional bandwidth and largest sky area survey at radio frequencies to date and calibrate the low-frequency flux density scale of the southern sky to better than 10 per cent. This paper presents details of the flagging, imaging, mosaicking and source extraction/characterization, as well as estimates of the completeness and reliability. All source measurements and images are available online. 1 This is the first in a series of publications describing the GLEAM survey results.
Research on the temperature control system based on fuzzy self-tuning PID

The temperature control system is increasingly playing an important role in industrial production. Recently, lots of researches have been investigated for the temperature control system based on various control strategies. The temperature control system based on fuzzy self-tuning PID is proposed in this paper. The new algorithm based on fuzzy self-tuning PID can improve the performance of the system. Also, it's fit for the complicated variable temperature control system. The simulation results show that the validity of the proposed strategy is more effective to control temperature.
“ Should I Stay Or Should I Go ” : Clash of Opinions in the Brexit Twitter Debate

Brexit, short for “British exit”, was one of the most important events to occur in recent British political and economic history. The EU referendum took place on the 23rd June, resulting in a win for the Leave campaign. The sentiment of both the Leave and the Remain campaigns clashed on mainstream media channels and social networking sites long before the day of the referendum; though still many are convinced that British voters were deceived — by one side or the other. With traditional opinion polls increasingly being doubted, the opportunity for alternative types of public opinion analysis arises. The micro-blogging site Twitter, on which users create 140-character messages to interact with each other, and which was a battleground for various stages of the referendum campaigning, seems to be the appropriate platform for such an analysis. This thesis is concerned with the analysis of Twitter debates surrounding the EU referendum through methods from network science as well as text data mining. We construct retweet and reply networks, in which nodes act as Twitter users whose interactions connect them to others by edges. To shed light on the structure of Brexit conversations, we investigate the presence of groups, called communities, within the networks. We examine users of these networks which are more influential, or central, than others and are thus perhaps more likely to be spreading information. We also apply methods from the field of text data mining on different types of textual data to learn about topics discussed in conversations around Brexit. We find that the methods we suggest give preliminary insights into the structure of the EU referendum debates and set the path for possible insightful, in-depth analyses of these Twitter conversations.
Serum Levels of Advanced Glycation End Products Are Associated with In-Stent Restenosis in Diabetic Patients

The formation of advanced glycation end products (AGEs), in various tissues has been known to enhance immunoinflammatory reactions and local oxidant stresses in long standing diabetes. Recently, AGEs have been reported to play a role in neointimal formation in animal models of arterial injury. We attempted to determine whether the serum levels of AGEs are associated with coronary restenosis in diabetic patients. Blood samples were collected from diabetic patients with coronary artery disease undergoing stent implantation and the serum levels of AGEs were analyzed by the fluorescent intensity method. The development of in-stent restenosis (ISR) was evaluated by a 6-month follow-up coronary angiography. A total of 263 target lesions were evaluated, in 203 patients. The ISR rate in the high-AGE (>170 U/ml) group (40.1%) was significantly higher than in the low-AGE group (≤170 U/ml) (19.6%) (p<0.001). Furthermore, multivariate analysis revealed that a high level of serum AGEs is an independent risk factor for the development of ISR (odds ratio, 2.659; 95% CI, 1.431-4.940; p=0.002). The serum levels of AGEs constitute an excellent predictive factor for ISR, and should be one of the guidelines for medical therapy and interventional strategy to prevent ISR in diabetic patients.
Statistical modelling on coordinates

Note This paper is a first draft of the principle of statistical modelling on coordinates. Several causes— which would be long to detail—have led to this situation close to the deadline for submitting papers to CODAWORK'03. The main of them is the fast development of the approach along the last months, which let appear previous drafts as obsolete. The present paper contains the essential parts of the state of the art of this approach from my point of view. I would like to acknowledge many clarifying discussions with the group of people working in this field in Girona, Barcelona, Carrick Castle, Firenze, Berlin, Göttingen, and Freiberg. They have given a lot of suggestions and ideas. Nevertheless, there might be still errors or unclear aspects which are exclusively my fault. I hope this contribution serves as a basis for further discussions and new developments.
Pensions and labour-market participation in the United Kingdom

Unlike most other European countries the United Kingdom's pension system is not well described by an analysis of the social-security element. For 30 years or more, around half the workforce has been covered by private occupational pensions. Something like half of the income of pensioners comes from non-socialsecurity sources, and this proportion is growing. Of the workforce in the mid-1990's, 75 percent are "contracted out" of the secondtier State Earnings Related Pension Scheme (SERPS) into private occupational or personal pensions. ' Partly as a result of these facts the United Kingdom also differs from many other countries in one other important respect: its state pension system is solvent. Tax rates necessary to pay for it are not predicted to rise despite the fact that the number of people over state retirement age is predicted to rise from 15.7 percent of the whole population to over 24 percent in 2050.2 In this paper we begin by describing the labor-market behavior of individuals around pension age. We also consider the coverage of the various parts of the social-security system. We go on to explain the structure of state pensions in the United Kingdom and compute the incentives for retirement that the structure creates, focusing on early retirement and the role of disability benefits, which are especially relevant for lower-skilled workers. We compare these incentives with those facing workers with private occupational schemes who now make up the mnajority of older workers close to retirement. The structure of incentives is found to match well with the observed patterns of labor-market participation in the data. L. The Labor-Market Behavior of Older Persons in the United Kingdom The labor-market behavior of older persons in the United Kingdom has been characterized by a severe fall in the participation of men. The rate of participation among recent cohorts falls sharply below 80 percent after the age of 50 and declines rapidly thereafter. In contrast the secular rise in the participation of women has resulted in a small upward trend in participation among women in the 55-60 age bracket, with participation rates approaching those for men
Relationship between single nucleotide polymorphisms in the 3’-untranslated region of the vascular endothelial growth factor gene and susceptibility to diabetic peripheral neuropathy in China

Introduction This study is to elucidate the relationship between a 936C/T mutation at the 3’-untranslated region of the human vascular endothelial growth factor (VEGF) gene and diabetic peripheral neuropathy (DPN). Material and methods All subjects recruited in this study were divided into DM (diabetes without neuropathy, retinopathy or nephropathy), DPN (diabetes with peripheral neuropathy only) and healthy control groups. The gene polymorphism was determined by polymerase chain reaction-restriction fragment length polymorphism, as well as other clinical methods including serum VEGF by ELISA. Results The C allele frequency and CC genotype frequency in the DPN group were higher than those in the NC group and DM group. The T allele frequency and CT+TT genotype (carrying the T allele) frequency in the DPN group were lower than those in the NC group (χ2 = 19.051 and 18.533, both p < 0.001) and DM group (χ2 = 11.117 and 11.156, both p = 0.001). However, there was no statistically significant difference in the three genotype (CC/CT+TT) frequencies and allele (C/T) frequencies between the DM group and the NC group. The multivariate logistic regression analysis showed that the levels of glycated hemoglobin (HbA1c), total cholesterol (TC), low density lipoprotein cholesterol (LDL-C) and plasma VEGF positively correlated with DPN, while the 936C/T gene polymorphism of VEGF negatively correlated with DPN. Conclusions Allele 936C of VEGF may serve as a genetic marker susceptible to DPN, while allele 936T may be a protective genetic marker of DPN.
How persistent is South Africa's inflation

An estimate of the persistence of inflation captures the long-run effects of a shock on inflation. A better understanding of the persistence of inflation can be useful for a variety of reasons. Firstly, a persistence measure can provide important information about the likely impact of shocks on the economy over time. This information can be extremely useful for the purpose of setting monetary policy, especially in a small, open economy like South Africa. Secondly, estimates of persistence over time can shed some light on the performance of monetary policy under different policy regimes. Finally, persistence estimates could assist in the identification of underlying inflationary pressures in the economy. This paper addresses these three issues with a reference to the South African experience. There are basically three main conclusions that emanate from the results in this paper. Firstly, there has been a significant decline in the persistence of inflation in South Africa. This decline occurred during the inflation targeting (IT) regime which suggests that IT provided a good anchor for inflationary expectations, despite the economy being subject to sizable external shocks during this period. Secondly, inflation at the disaggregate level is lower than at the aggregate level. This implies that the impact of external shocks on aggregate inflation will depend on each commodityâ€™s level of inflation persistence as well as its weight in the consumption basket. Finally, a core measure of inflation, which considers both the persistence of inflation at the disaggregate level as well as the weight of the commodity in the consumption basket, does a better job in identifying the underlying inflationary pressures in the economy when compared to the other more readily available core measures. This measure may thus be a useful addition to the range of indicators considered for policy-making purposes.
Creating an ITIL inspired Incident Management approach: Roots, response, and results

Incident Management is a key element of supporting any system. For Internet-based applications this function requires integration of staff, process, and tools to manage responses to integrated system events including carrier network outages or performance impacts, hosted server and infrastructure failures, application errors, and mis-configurations. A detailed description of the origin of the Incident Management approach at Wolters Kluwer is discussed, including a summary of the ITIL (Information Technology Infrastructure Library) guidance on which it is based, focusing on the custom process and tools developed to realize an improved approach over time, and the results achieved.
Campaign Spending and Electoral Competition: Towards More Policy Relevant Research1

Abstract There is a long-standing scholarly literature on the electoral effects of campaign spending. Nevertheless, the academic research offers only limited guidance for policy makers interested in campaign finance reform. In part, this is because existing studies have focused narrowly on some vexing statistical issues. But it is also because political scientists have not devoted enough effort to conducting evaluation studies of how regulatory policies impact the intermediate goal of competition, let alone the ultimate policy goals of reduced corruption, increased citizen participation, and improved public policy. Consequently, there is a great need for updated and improved analyses of the treatment effect of campaign spending on political competition in a variety of electoral contexts, but an even greater need for the application of modern evaluation methods to the more basic question of whether campaign finance reforms “work” as advertised.
Remembering and imagining differentially engage the hippocampus: A multivariate fMRI investigation

It has been proposed that imagining the future depends on the ability to retrieve episodic details from past experiences in order to recombine them into novel possible experiences; consequently, the processes of remembering and imagining rely on similar neural substrates, including the hippocampus. We used fMRI and both univariate and multivariate analysis techniques to test this prediction. Unbiased univariate analysis did not reveal differences in the hippocampus between remembering and imagining; however, multivariate analyses revealed evidence that patterns of activity within the hippocampus distinguish between remembering and imagining. Thus, while the hippocampus seems to be involved in both remembering the past and imagining the future, the pattern of activity within the hippocampus distinguishes between these two different tasks.
Interspike Interval Statistics in the Stochastic Hodgkin-Huxley Model: Coexistence of Gamma Frequency Bursts and Highly Irregular Firing

When the classical Hodgkin-Huxley equations are simulated with Na- and K-channel noise and constant applied current, the distribution of interspike intervals is bimodal: one part is an exponential tail, as often assumed, while the other is a narrow gaussian peak centered at a short interspike interval value. The gaussian arises from bursts of spikes in the gamma-frequency range, the tail from the interburst intervals, giving overall an extraordinarily high coefficient of variationup to 2.5 for 180,000 Na channels when I 7Acm2. Since neurons with a bimodal ISI distribution are common, it may be a useful model for any neuron with class 2 firing. The underlying mechanism is due to a subcritical Hopf bifurcation, together with a switching region in phase-space where a fixed point is very close to a system limit cycle. This mechanism may be present in many different classes of neurons and may contribute to widely observed highly irregular neural spiking.
Aluminum surface treatment using three different plasma-assisted dry chemical processes

Aluminum plate surfaces having hydrophilic and corrosion-resistant properties are often required for various mechanical parts or systems. The conventional process for treating aluminum surfaces employs a series of wet processes: grease or organic compounds removal followed by drying, chromate process followed by drying, and finally chemical treatment to achieve hydrophilic property. However, the use of chromium (Cr/sup +6/) in the chromate process creates an environmental problem. A simple and economic dry process was investigated to replace the conventional aluminum surface treatment, i.e., nonthermal plasma followed by the chemical process. Among several nonthermal plasma processes, two types of plasma jets (60-Hz ac plasma and RF plasma) and the pulsed-corona plasma were investigated. The adhesive characteristics between the aluminum surface and the corrosion-resistant chemical were evaluated by the accelerated corrosion test, i.e., copper-accelerated acetic acid spray (CASS) test. RF plasma with more than 30 s of exposure time followed by the epoxy-type corrosion-resistant coating showed a superior performance over the conventional chromate wet process. Pulsed corona process was nearly equal to the conventional process. Therefore, the conventional wet chemical process for aluminum treatment can be replaced by the newly developed plasma-assisted chemical process.
Space-time information propagation in mobile ad hoc wireless networks

Gupta and Kumar (1999) have shown that density increases wireless capacity. Grossglauser and Tse (2001) have shown that mobility also increases capacity. We quantify more precisely these properties by setting up the general laws that the propagation path must satisfy in the presence of traffic flow density. Introducing the time constraint in packet delivery, we generalize to a space-time problem with mobile networks.
Sustainable livelihoods to adaptive capabilities: a global learning journey in a small state, Zanzibar

This thesis takes global learning out of the formal setting of a Northern classroom to a rural community setting in the Global South as a social learning process. It begins with a critical reflection of a large EU project to develop a global learning programme as a Global North South initiative. The focus narrows to Zanzibar, a small island state, to critically reflect on the delivery of the programme. And then further to focus on the global social learning and change that occurred in a rural community setting in the north of the island. Through participatory action research, I investigate the relevance of global learning as a social learning process, how norms and rules are shaped within a community setting and how these enable social change towards sustainable livelihoods. The thesis splices the intersection between critical and social theories of learning and engagement, to include critical social theories of Habermas (1984) and Wals (2007); critical race theories of Giroux (1997) and Said (1994) and distributive justice and entitlements theories of Sen (1997) and Moser (1998). It demonstrates the importance of dissonance and a safe space for deliberative dialogue, to be able to consider the global pressures and forces on local realities as the precursor to social change towards sustainability. I conclude by relating the learning from this small island state to the wider world and the current discourse on quality of education in a community development context.
CONCEPTUAL CLARIFICATION OF STRATEGIC HUMAN RESOURCE MANAGEMENT AND THE WAY IT IS IMPLEMENTED

Strategic human resource management issue is treated by many authors, following this paper we will try to clarify, to emphasize the role of this concept and how it should be implemented in domestic comapniile. Defining the concept of strategic human resource management, requires perfect understanding of human resource management, the concept referring to the philosophies, policies, procedures and practices related with people management in an organization. The main purpose of human resource management is to add value not only to the process of obtaining, but also to the products and services themselves, together with increasing the level of work quality and creating a better standard of living for the employees.The human resources can be considered economical resources and thus a strategy generating costs opprotunities is required. Even though it is very relevant for the huuman resources positions, the HR departments do not welcome the concept of „cost opportunity” probably because the individuals in managing positions consider themselves working with people and not with money or maybe they are specialists regarding the job positions related to the personnel and not regarding the decisions with direct impact on the business. The actual reality proves that the human resource „faults” can largely generate cost opportunities within a company.
Shifted-Delta MLP Features for Spoken Language Recognition

This letter presents our study of applying phoneme posterior features for spoken language recognition (SLR). In our work, phoneme posterior features are estimated from a multilayer perceptron (MLP) based phoneme recognizer, and are further processed through transformations including taking logarithm, PCA transformation, and appending shifted delta coefficients. The resulting shifted-delta MLP (SDMLP) features show similar distribution as conventional shifted-delta cepstral (SDC) features, and are more robust compared to the SDC features. Experiments on the NIST LRE2005 dataset show that the SDMLP features fit well with the state-of-the-art GMM-based SLR systems, and SDMLP features outperform SDC features significantly.