Miracle-dz/UniFAR-FTUs huggingface dataset:
Mutations in RD3 are associated with an extremely rare and severe form of early onset retinal dystrophy.

PURPOSE To identify the underlying mutation and describe the phenotype in a consanguineous Kurdish family with Leber's congenital amaurosis (LCA)/early onset severe retinal dystrophy (EOSRD). METHODS Members of the index family were followed up to 22 years by ophthalmological examinations, including best corrected visual acuity (BCVA), Goldmann visual field (GVF), two-color-threshold perimetry (2CTP) and Ganzfeld electroretinogram (ERG), fundus photographs, fundus autofluorescence (FAF), and optical coherence tomography (OCT). After excluding seven of nine known LCA/EOSRD genes in the index patient, linkage analysis was performed in the family using a microarray followed by microsatellite fine mapping and direct sequencing of candidate genes. RD3 was screened by direct sequencing of 85 independent patients with LCA/EOSRD presenting with a BCVA ≥ 1.0 LogMAR before the age of 2 years to assess the prevalence of RD3 mutations in LCA/EOSRD. Since RD3 and RetGC1 have a functional relation, study authors screened for a modifying effect of RD3 mutations in 17 independent patients with mutations in GUCY2D. RESULTS BCVA was severely reduced from the earliest examinations (as early as 3 months), never exceeding 1.3 LogMAR. The disease presented as cone-rod dystrophy with dystrophic changes in the macula and bone spicules in the periphery on progression. Linkage analysis narrowed the region of interest towards the LCA12 locus. Direct sequencing of RD3 revealed a homozygous nonsense mutation (c.180C > A) in all affected members tested. Screening of additional unrelated LCA/EOSRD patients revealed only polymorphisms in RD3. CONCLUSIONS This is the second family reported so far with mutations in RD3. Mutations in RD3 are a very rare cause of LCA associated with an extremely severe form of retinal dystrophy.
Accounting relevance and speculative intensity: empirical evidence from Greece

Purpose - The aim of this paper is twofold: first, it aims to examine the relevance of earnings and book values on stock prices, and second, to test for the effect of speculative intensity on the relevance of accounting information between 1995 and 2004. Design/methodology/approach - The data were collected from a sample of 101 non-financial firms listed at the Athens Stock Exchange over the period 1996-2004 and were analyzed using OLS regression models. Findings - Results indicated that book values are not relevant when they are considered solely, but both earnings and book values are more relevant when they are simultaneously included in the model. Finally, taking into consideration the effect of speculative intensity, we observed that it has a positive and significant effect on stock prices yet the value relevance of earnings and book values has not changed even after controlling for speculation. Practical implications - The findings can be used by analysts and capital market researchers since both must bear in mind the fact that the quality of the capital market and the quality of the stock prices are not constant over time. Consequently, the classical valuation models are misspecified and could yield significant bias if speculative intensity is not taken under consideration. Originality/value - The findings provide a further insight on the issue of accounting relevance within the context of an emerging capital market like Greece. According to our knowledge this is the first study which concerns speculation as an important factor of accounting value relevance in an European country.
Extent of MRI delayed enhancement of myocardial mass is related to right ventricular dysfunction in pulmonary artery hypertension.

OBJECTIVE The purpose of our study was to assess the presence and extent of delayed contrast enhancement of ventricular myocardium in pulmonary artery hypertension. SUBJECTS AND METHODS Fifteen patients (age, 45.6 +/- 13 years; 13 New York Heart Association class III) with pulmonary artery hypertension (11 idiopathic, four systemic sclerosis) were studied. All patients had undergone a comprehensive diagnostic workup, and pulmonary artery hypertension (mean pulmonary artery pressure, 54 +/- 16 mm Hg) was confirmed by cardiac catheterization. Cardiac MRI was performed on a 1.5-T scanner to determine ventricular volumes and mass. Delayed contrast enhancement of a mass was seen 10-20 minutes after the i.v. injection of 0.2 mmol/kg of gadopentetate dimeglumine using an inversion recovery gradient-echo sequence. RESULTS All patients showed delayed contrast enhancement at the insertion points of the right ventricular free wall to the interventricular septum (15 inferior, 13 anterior). The mean weight of the delayed contrast-enhanced myocardial mass was 3.1 +/- 1.9 g (size range, 0.3-3.9% of the total myocardial mass). The extent of the delayed contrast-enhancing myocardium was inversely related to the right ventricular ejection fraction (r = -0.63, p = 0.001), right ventricular stroke volume (r = -0.67, p = 0.006), and right ventricular end-systolic volume index (r = -0.51, p = 0.05) but not to any invasively measured hemodynamic index or N-terminal pro brain natriuretic peptide. CONCLUSION Myocardial delayed contrast enhancement occurs frequently in patients with severe symptomatic pulmonary artery hypertension and is inversely related to measures of right ventricular systolic function.
Note on the Productivity Convergence of Airports in China

The present paper uses a Malmquist index measure of productivity growth to study the productivity convergence of 25 airports in China during the period 1995–2004. By decomposing the Malmquist index, we find evidence for the convergence of the efficiency level of airports, but not the productivity and technology levels. These results imply that the more productive airports are pushing the frontiers of technology faster by adopting new technology, and that the relative efficiency level of the laggards increases quickly and catches up with the efficiency level of the leaders over time.
Multi-attribute sparse representation with group constraints for face recognition under different variations

A novel multi-attribute sparse representation enforced with group constraints is proposed in this paper. Data with multiple attributes can be represented by individual binary matrices to indicate the group properties for each data sample. Then, these attribute matrices are incorporated into the formulation of l1-minimization. The solution is obtained by jointly considering the data reconstruction error, the sparsity property as well as the group constraints, thus making the basis selection in sparse coding more efficient in term of accuracy. The proposed optimization formulation with group constraints is simple yet very efficient for classification problems with multiple attributes. In addition, it can be derived into a modified sparse coding form so that any l1-minimization solver can be employed in the corresponding optimization problem. We demonstrate the performance of the proposed multi-attribute sparse representation algorithm through experiments on face recognition with different kinds of variations. Experimental results show that the proposed method is very competitive compared to the state-of-the-art methods.
Experimental Sealed Bid First Price Auctions with Directly Observed Bid Functions

Nine groups of three subjects each repeatedly interacted in sealed bid first price auctions for fifty periods. In every period each subject specified a piecewise linear bid function shown on the computer screen. Then random values were drawn for each subject and bids were determined according to the bid functions. The object was sold to the highest bidder at the price of his bid. Many bid functions were almost linear but a substantial fraction of them was non-monotonous. The subjects changed their bid functions considerably during the fifty periods of play. The direction of change exhibits marked regularities which can be explained by a "learning direction theory". The bid at last period's value tends to be lowered or not to be increased after the object has been obtained; it tends to be increased or not to be lowered if the object was not obtained but the price was below the value. A typicity analysis shows that subjects conform to the predictions of learning direction theory the more strongly the more typical they are.
A Regime‐Switching Approach to Modeling Rental Prices of U.K. Real Estate Sectors

This article uses regime-switching models of the threshold type to analyze the adjustment process of rental prices for three U.K. commercial real estate sectors over the period 1974-2008. The nonlinear models outperform their linear counterparts in in-sample fit. Their out-of-sample forecasting ability is better whenever the corresponding linear models contain a significant amount of neglected nonlinearity. Regime switches are triggered when the growth rates of rental price exceed certain threshold levels. For the industrial and retail sectors such regime switches occur in situations of strong excess demand, for the office sector they occur when there is strong excess supply.
The 'legalisation' of education : a study of New South Wales teachers and their professional development needs in the area of law

This study is concerned with the legal knowledge held by teachers of New South Wales Government schools and the need for continued professional development in this area. The study commenced in 1997 and primary research concluded in 2000. The Australian experience seems to be mirroring international experiences, particularly those of the United States of America, which have seen an increase in the legalisation of the field of education. From this increase there emerged a need for a systematic programme of instruction to address the problem of a lack of legal knowledge held by teachers in order to adequately perform their professional duties as teachers and protect themselves and their employer from potential legal liabilities. The main purposes of this study were to determine the degree of legal knowledge that teachers in the State of New South Wales possessed, the need for the development of a course of instruction to address the perceived problem of a lack of legal knowledge and to investigate the methods available to address this problem. The study then proceeded to develop and trial a systematic curriculum designed to provide a background for teachers to education and the law. This was a multimethod ethnographic exploratory study that integrated aspects of both quantitative and qualitative research paradigms. An initial focus group interview schedule was developed and then trialled with a group of teachers in order to gauge their experiences and knowledge of the law. This interview schedule was then refined and trialled with a larger sample of teachers drawn from
Economic appraisal of rural roads : simplified operational procedures for screening and appraisal

This paper suggests simple operational approaches to the screening and economic appraisal of road components of rural roads and development projects. It is based on a review of appraisal and preparation documents of 15 "traditional" bank rural roads projects and 5 rural development projects with rural road components, as well as relevant literature. The objective is to simplify screening and appraisal methods without reducing their reliability to unacceptable levels. The proposed screening methods are based on the principles of discriminant analysis and a process of finding a quick and rough estimation of an economic return. (TRRL)
Poverty and Banking Exclusion in Mexico: How can remittances and technology contribute to improve access?

The empowerment of the poor through cell phones is this paper’s central theme. There already exist a number of cases in the world where such empowerment has already occurred in the financial sector through the usage of cell phones: the cases provided throughout the paper are Kenya, the Philippines and South Africa. There exist many reasons why it is imperative to facilitate access to the financial system for the entire pyramid of the population. To achieve financial inclusion in the remittances industry, the authors suggest an M-banking like solution for remittances transfer. M-banking offers the advantage of having a very low cost structure – making it lucrative for banks to target the rural poor. Poverty is an insidious problem in Mexico. A large share of the population has to live on less than US$2 a day. In rural areas, and particularly in the Southern states, the issue is even more pressing. High levels of poverty lead to high rates of migration towards the North. Augmented migration in turn results in remittances inflows, which have reached unprecedented levels in Mexico with about US24bn in 2006. But immigrants have not fully capitalized their remittances because they are outside the banking system and pay high costs of transfers. New and cheap channels for transferal are available, but only for the banked. In Mexico between 20 or 30 percent of the population do have a bank account. However, cell phone penetration is about 45 percent – about twice as high as the level of bank account ownership. Consequently, given cell phones’ increased processing power and the high penetration rates of cell phones, they are the obvious means to have people take part in the financial sector. We show in our econometric study that remittances promote savings accounts, but at the same time have an ambiguous effect on them through increased cell phone penetration. By creating virtual mobile bank accounts, banks can leverage the relatively high cell phone penetration across the income pyramid, to bank Mexico’s population – while benefiting from additional profitable business. Given remittances’ savings increasing nature, starting with remittances’ recipients is the logical and most powerful tipping point of the process. Our study finds that banks ignore the potential of the mobile banking market, including remittances transfers over cell phones. We suggest a number of policies to be implemented to enable the prospering of the mobile financial industry.
Forecasting system energy demand

This paper presents the results of the Electric Power Research Institute Short Range Forecasting Project (EPRI-SRF) performed by the Load Forecasts Department, Economics and Forecasts Division of Ontario Hydro, Ontario, Canada. In this study a variety of short-range forecasting techniques are applied to Ontario Hydro monthly data on total system energy demand. These techniques are available in a software package (FORECAST MASTER) developed for EPRI by two consultants—Scientific Systems, Inc. (SSI) and Quantitativ Economic Research, Inc. (QUERI). The methods used for this study were the univariate Box-Jenkins method, the multivariate state-space method, Bayesian vector autoregression and autoregress ve econometric regression. A comparison of the models developed show that the econometric models perform the best overall. The state-space models are more suitable for very short-term (one-step ahead) forecasts. Although the Box-Jenkins method has the advantage of simplicity in terms of estimation and data requirement, its performance was not as good as that of the others. Bayesian vector autoregresson results indicate that this program needs some modification for monthly data.
Social security expenditures and economic growth: A heterogeneous panel application

Purpose – The purpose of this paper is to re-examine the long-run co-movement and causal relationship between GDP and social security expenditures. Design/methodology/approach – The paper uses panel data unit root tests and panel cointegration tests, as well as estimation techniques appropriate for heterogeneous panels such as fully modified OLS. Data are employed on 12 Asian countries from 1972 to 2000. Findings – The cointegration test results show strong evidence in favor of the existence of a long-run equilibrium cointegrating relationship between GDP, capital stock and social security expenditures after allowing for heterogeneous country effects. Regarding the panel-based error correction model and the Granger causality test, there are long-run, bi-directional causal linkages between social security expenditures and economic growth. In addition to the robust test, they display similar results. Originality/value – The paper shows that in every moment, economic growth must be based in the social welfare policy contiguously, and the economic growth process can allow the social welfare policy to proceed contiguously
Optimal bank asset and liability management with financial futures

This paper presents a model for optimal bank asset and liability management with financial futures. This model is a multiperiod linear programming model based on Markowitz portfolio theory. Given the bank's initial position, its economic forecasts, and the constraints under which it operates, the model can help a bank's senior executives determine the current and expected future balance sheet composition and financial futures position which will minimize the bank's operating risks and which will meet the bank's expected profits goal with the minimum possible profits risk. By parametrically varying the expected profits goal, the model will generate the set of risk‐return efficient decisions. Bankers need then examine only the set of efficient decisions to choose their optimal solution. A simplified example is used to illustrate the application of our model and to demonstrate that banks that use financial futures in asset and liability management can obtain better results than banks that do not use financial futures.
The Reelection Debate in Latin America

Abstract The debate over presidential reelection reappeared in Latin America in the last decade, and promises to continue in the years ahead. Arguments in favor contend that the possibility of immediate reelection increases politicians' responsiveness to citizen demands and allows voters the freedom to retain popular incumbents. Arguments against emphasize the danger of abuse of power by incumbents who seek to prolong their tenure. This article illustrates the parallels between these arguments and those made historically regarding the issue. It also suggests that the means by which provisions to allow reelection are adopted can provide valuable signals of their consequences.
Genome-wide nested association mapping of quantitative resistance to northern leaf blight in maize

Quantitative resistance to plant pathogens, controlled by multiple loci of small effect, is important for food production, food security, and food safety but is poorly understood. To gain insights into the genetic architecture of quantitative resistance in maize, we evaluated a 5,000-inbred-line nested association mapping population for resistance to northern leaf blight, a maize disease of global economic importance. Twenty-nine quantitative trait loci were identified, and most had multiple alleles. The large variation in resistance phenotypes could be attributed to the accumulation of numerous loci of small additive effects. Genome-wide nested association mapping, using 1.6 million SNPs, identified multiple candidate genes related to plant defense, including receptor-like kinase genes similar to those involved in basal defense. These results are consistent with the hypothesis that quantitative disease resistance in plants is conditioned by a range of mechanisms and could have considerable mechanistic overlap with basal resistance.
Albumin corona on nanoparticles – a strategic approach in drug delivery

Abstract Nanomaterials have been used widely for delivery of therapeutic agents. Protein–nanoparticle (NP) complexes have gained importance as vehicles for targeted drug delivery due to increased ease of administration, stability and half-life of drug, and reduced toxic side effects. Designing of phospholipid–bovine serum albumin (BSA) complexes and stealth NPs with BSA has paved the way for drug delivery carriers with prolonged blood circulation times. Preformed albumin corona has shown to decrease non-specific association and thereby reduce the clearance rate. Albumin corona has enabled the localization of drug carriers in specific tissues such as liver and heart, thus regulating biodistribution. Tailored albumin–NP conjugates have also enabled controlled degradation of NP and drug release. However, the binding of albumin with NP is associated with conformational and functional modulations in protein as observed with silver, gold and superparamagnetic iron oxide NPs. In this review, we highlight the various potential albumin–NP hybrids as nano drug carriers.
Spatial spectrum sensing for wireless handheld terminals: design challenges and novel solutions based on tunable parasitic antennas [Dynamic Spectrum Management]

This article addresses the major design challenges of antenna systems intended for cognitive transceivers under compactness constraints. The first part of the article provides an overview of the design challenges with regard to sensing over different frequencies and angular directions, referred to as spatial spectrum sensing. The second part describes a novel approach based on parasitic antenna theory aiming at making spatial spectrum sensing feasible for portable lightweight terminals. The idea is to replace the wideband antenna required for sensing over a large bandwidth by a tunable narrowband antenna for both sensing and communication purposes. The flexibility of the proposed antenna system lies in its capability to scan both the frequency and spatial resource dimensions simultaneously via a single RF chain within a miniaturized antenna system. This is done by properly tuning a set of reactive loads connected to a group of parasitic elements closely coupled to the driven (active) element. By doing so, the operational frequency subband leaps to another subband (frequency tuning). Moreover, at every subband, circular permutations of the reactive loads rotate the narrowband beam pattern to different angular positions, giving the cognitive transceiver the capability of sensing over various segments of the space.
SOUTH AFRICA'S TRANSITION TO A CONSOLIDATED BUDGET

The size of South Africa's fiscal stimuli, whether intended or not, has helped to avert negative consequences of the global financial downturn. With the economic cycle turning again, consolidation of deficits and a reduction of the level of debt are again the focus of policymakers. These outcomes are generally achieved by either increasing tax rates or cutting spending (discretionary fiscal policy), whereas an alternative option is to allow automatic stabilisers to consolidate budgets. This study attempts to answer whether cyclical factors or discretionary policy minimise output volatility and which one of the two presents a better policy option regarding uncertainty in real economic recovery. For this purpose, a small open-economy gap model is built using South African data, where the budget deficit is endogenised by way of a fiscal policy “rule. ” Sensitivity analyses and robustness checks are carried out using a structural vector autoregression. Given the estimates of both the automatic stabilisers as well as the components of discretionary fiscal policy, we are able to obtain impact multipliers on output and conduct scenario testing for optimal fiscal policy response towards fiscal consolidation as well as debt sustainability.