Miracle-dz/UniFAR-FTUs huggingface dataset:
The mutant genotype is the main determinant of the metabolic phenotype in phenylalanine hydroxylase deficiency.

Phenylketonuria and mild hyperphenylalaninemias are allelic disorders caused by mutations in the phenylalanine hydroxylase (PAH) gene. Following identification of the disease-causing mutation in 11 PAH-deficient patients, we tested the activity of the mutant gene products in an eukaryotic expression system. Two mutations markedly reduced PAH activity (A259V and L333F), one mutation mildly altered the enzyme activity (E390G), while the majority of mutant genotypes reduced the in vitro expression of PAH activity to 15-30% of controls. Comparing the predicted residual activity derived from expression studies to the clinical phenotypes of our PAH-deficient patients, we found that homozygosity for the L333F and E390G mutations resulted in severe and mild PAH deficiencies, respectively, both in vivo and in vitro, while compound heterozygosity (L333F/E390G) resulted in an intermediate dietary tolerance. Similarly, in vitro expression studies largely predicted dietary tolerance in compound heterozygotes for the A259V/IVS12nt1 (typical PKU), A259V/A403V, G218V/I65T, and G218V/R158Q mutations (mild variants). Taken together, these results support the view that expression studies are useful in predicting residual enzyme activity and that the mutant genotype at the PAH locus is the major determinant of metabolic phenotype in hyperphenylalaninemias.
Income Distribution, Pre-Distribution & Re-Distribution Latest data for the Republic of Ireland

SUMMARY The latest income distribution data for Ireland comes from the 2011 CSO Survey on Income and Living Conditions (SILC), released in 2013. This Research inBrief uses that data to examine the distribution of income pre and post redistribution. While policy tends to focus on the level of post-distribution income inequality (after taxes and welfare payments), it is important to consider the starting point for policy, the pre-distribution structure of direct (market) income. This paper outlines the structure of Ireland’s income distribution across households assessing direct income (earnings), gross income (earnings plus welfare) and disposable income (earnings plus welfare minus income taxes and social insurance). It also quantifies the impact of Ireland’s redistribution system on income inequality.
Unexpected Shortfalls of Expected Shortfall: Extreme Default Profiles and Regulatory Arbitrage

The purpose of this paper is to dispel some common misunderstandings about capital adequacy rules based on Expected Shortfall. We establish that, from a theoretical perspective, Expected Shortfall based regulation can provide a misleading assessment of tail behavior, does not necessarily protect liability holders’ interests much better than Value-at-Risk based regulation, and may also allow for regulatory arbitrage when used as a global solvency measure. We also show that, for a value-maximizing financial institution, the benefits derived from protecting its franchise may not be sufficient to disincentivize excessive risk taking. We further interpret our results in the context of portfolio risk measurement. Our results do not invalidate the possible merits of Expected Shortfall as a risk measure but instead highlight the need for its cautious use in the context of capital adequacy regimes and of portfolio risk control.
Do Introduction Programs Affect the Probability of Immigrants getting Work

Many immigrants who come to Sweden are offered an introduction program. This is supposed to allow the individual to develop the skills he or she needs to be able to enter the Swedish labor market. With a unique Swedish dataset, containing information on introduction activities, we investigate the impact of different introduction activities on the immigrants’ employment probability, in a short-run perspective. Our basic findings are that some activities, such as labor market practice, have a positive effect, while other activities do not seem to have any impact or even negative effect on the individuals’ probabilities of getting a job.
High-Intensity Discharge Lamps With High Color Rendering Indices Based on Molecular Radiation of Indium Iodide

This paper deals with mercury-free high-intensity discharges based on indium iodide, that are driven by guided microwaves at 2.45 GHz. The influences of different additives to the lamp on the luminous efficacy as well as on the color rendering index will be shown. Furthermore, the spectral output of pure indium iodide lamps, using argon as auxiliary gas, will be explained by a combination of indium line radiation and molecular emission of the indium iodide molecules. A theoretical model for the molecular emission is shown, which uses the semiclassical description of molecular radiation.
Transforming Payment Choices By Doubling Fees on the Illinois Tollway

On January 1, 2005, Illinois doubled the highway toll for travelers paying with cash, but kept the price unchanged for those paying electronically. This paper combines a theoretical model of payment choice with empirical analysis based on this rare natural experiment of differential pricing depending on the method of payment: cash versus electronic payment. An actual response to a price change allows the authors to estimate the sensitivity of consumer payment demand to prices.
Molecular and morphologic analyses of expression of ESX1L in different stages of human placental development

The mRNA expression of the ESX1L gene was analyzed by RT‐PCR and in situ hybridization in human normal cytogenetically placentas, of different gestational ages. Our RT‐PCR analysis showed that ESX1L mRNA is expressed from 5 weeks of gestation until term, suggesting a role not only in trophoblast differentiation but also in the maintenance of the villi and microvasculature. We also observed, by in situ hybridization, that ESX1L mRNA is expressed by cytotrophoblast from chorinic plate, syncytiotrophoblast and stromal cells of all terminal, intermediate and stem villi of term placentas. ESX1L mRNA expression was more pronounced in trophoblast cells of terminal villi than in intermediate and sterm villi. In conclusion, ESX1L is expressed during all stages of placental development and is localized to sparse areas of trophoblast in terminal villi in association with cytotrophoblastic cells.
Economic Valuation of Coastal and Marine Resources: Bohol Marine Triangle, Philippines

This article provides results on the net benefits generated from the natural resources in the Bohol Marine Triangle (BMT) in the Philippines. The BMT spans over 112,000 ha and its coastal ecosystems are rich in biodiversity and provide economic opportunities to the coastal communities. With a 10% discount rate, the accumulated total net benefits for the BMT resources over a 10-year period is US$11.54 million. Tourism and the municipal fisheries are the most important direct use values of the coastal and marine resources of the BMT accounting for 44% and 39% of the total net benefits. Annual revenues attributed to ecosystems were as follows: coral reefs, US$1.26 million; beach/intertidal area, US$1.12 million; marine waters, US$646,501; mangrove, US$239,561; and seagrass, US$105,990. The large market values indicate the dependence of the local community on the BMT coastal and marine resources. In the same way, non-market values show the important life-support functions of coastal and marine ecosystems. The net benefits reflect the magnitude of potential losses due to improper management of coastal and marine resources in the BMT. This valuation highlights the importance of the coastal services to the BMT economy and draws attention to the benefits the local stakeholders derive from BMT coastal resources. Policy measures can now take into account these values to justify a sufficient investment in coastal management efforts to sustain the flow of coastal services in the interest of current and future generations.
E‐cash issues: electronic cash is not just about technology

Discusses the limitations of cash as a means of exchange and looks to the digital networks of the future to exploit the new technologies of e‐cash. Highlights the convenience and increasingly widespread use of digital money in business‐to‐consumer and business‐to‐business transactions. The emzergence of common standards and pilot projects on a variety of Internet‐based payment mechanisms by companies such as IBM, Digital, Barclays and Unisource is considered. Looks at the social issues surrounding digital money and the impact of lowering the entry costs in the money business. Options for domain regulation of fiat and private currencies are discussed, concluding that the central issue rests on whether government or the market controls the issuing of e‐cash.
σ-1 Receptor Modulation of Acid-Sensing Ion Channel a (ASIC1a) and ASIC1a-Induced Ca2+ Influx in Rat Cortical Neurons

Acid-sensing ion channels (ASICs) are proton-gated cation channels found in peripheral and central nervous system neurons. The ASIC1a subtype, which has high Ca2+ permeability, is activated by ischemia-induced acidosis and contributes to the neuronal loss that accompanies ischemic stroke. Our laboratory has shown that activation of σ receptors depresses ion channel activity and [Ca2+]i dysregulation during ischemia, which enhances neuronal survival. Whole-cell patch-clamp electrophysiology and fluorometric Ca2+ imaging were used to determine whether σ receptors regulate the function of ASIC in cultured rat cortical neurons. Bath application of the selective ASIC1a blocker, psalmotoxin1, decreased proton-evoked [Ca2+]i transients and peak membrane currents, suggesting the presence of homomeric ASIC1a channels. The pan-selective σ-1/σ-2 receptor agonists, 1,3-di-o-tolyl-guanidine (100 μM) and opipramol (10 μM), reversibly decreased acid-induced elevations in [Ca2+]i and membrane currents. Pharmacological experiments using σ receptor-subtype-specific agonists demonstrated that σ-1, but not σ-2, receptors inhibit ASIC1a-induced Ca2+ elevations. These results were confirmed using the irreversible σ receptor antagonist metaphit (50 μM) and the selective σ-1 antagonist BD1063 (10 nM), which obtunded the inhibitory effects of the σ-1 agonist, carbetapentane. Activation of ASIC1a was shown to stimulate downstream Ca2+ influx pathways, specifically N-methyl-d-aspartate and (±)-α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid/kainate receptors and voltage-gated Ca2+ channels. These subsequent Ca2+ influxes were also inhibited upon activation of σ-1 receptors. These findings demonstrate that σ-1 receptor stimulation inhibits ASIC1a-mediated membrane currents and consequent intracellular Ca2+ accumulation. The ability to control ionic imbalances and Ca2+ dysregulation evoked by ASIC1a activation makes σ receptors an attractive target for ischemic stroke therapy.
Measuring political protest in Western Europe: assessing cross-national equivalence

This article tests the cross-national equivalence of the political protest scale, as developed by Barnes and Kaase, in 20 Western European countries using a battery of items included in the fourth wave of the European Values Study. The scale measuring the concept of political protest is widely used, but no evidence of cross-country equivalence has yet been provided in the literature. The article illustrates the concept of political protest, the relationship between concept formation, operationalization, and measurement equivalence, and the possible consequences of a lack of equivalence. It is argued that comparative research may be threatened by a lack of measurement equivalence. The spread of international surveys eases comparative designs, but at the same time enlarges the chances that we compare what is not actually comparable. The article then outlines an empirical strategy to assess the political protest scale's measurement equivalence. To assess cross-country equivalence, Mokken Scale Analysis, a nonparametric scaling method within the family of Item Response Theory models, is used. This has been shown to work better than Confirmatory Factor Analysis when dealing with dichotomous and polytomous items forming ordinal scales. The results show that the cross-country equivalence of the political protest scale depends on the type of measure the scholar wishes to build and use.
Open-Market Share Repurchase Programs and Bid-Ask Spreads on the NYSE: Implications for Corporate Payout Policy

Abstract This study analyzes bid-ask spreads surrounding announcements of open-market share repurchase programs for a sample of 248 announcements of repurchase programs by NYSE firms over the period January 1984 through June 1988. The sample includes 158 announcements of new programs and 90 announcements regarding continuations of already existing programs. Contrary to the theory that spreads increase surrounding the announcement of open-market share repurchase programs, with both univariate and multivariate tests that control for changes in volume, changes in stock price volatility, and changes in the level of stock price, we find no evidence of an increase in spreads surrounding announcements of open-market share repurchase programs.
The CO-H2 Conversion Factor in Disc Galaxies and Mergers

Relating the observed CO emission from giant molecular clouds (GMCs) to the underlying H2 column density is a long-standing problem in astrophysics. While the Galactic CO–H2 conversion factor (XCO) appears to be reasonably constant, observations indicate that XCO may be depressed in high surface density starburst environments. Using a multiscale approach, we investigate the dependence of XCO on the galactic environment in numerical simulations of disc galaxies and galaxy mergers. XCO is proportional to the GMC surface density divided by the integrated CO intensity, WCO, and WCO is related to the kinetic temperature and velocity dispersion in the cloud. In disc galaxies (except within the central ∼ kpc), the galactic environment is largely unimportant in setting the physical properties of GMCs provided they are gravitationally bound. The temperatures are roughly constant at ∼10 K due to the balance of CO cooling and cosmic ray heating, giving a nearly constant CO–H2 conversion factor in discs. In mergers, the velocity dispersion of the gas rises dramatically during coalescence. The gas temperature also rises as it couples well to the warm (∼50 K) dust at high densities (n > 104 cm−3). The rise in velocity dispersion and temperature combine to offset the rise in surface density in mergers, causing XCO to drop by a factor of ∼2–10 compared to the disc simulation. This model predicts that high-resolution Atacama Large Millimeter/submillimeter Array observations of nearby ultraluminous infrared galaxies should show velocity dispersions of 101–102 km s−1, and brightness temperatures comparable to the dust temperatures.
New tumour necrosis factor inhibitors for rheumatoid arthritis: are there benefits from extending choice?

Certolizumab pegol (UCB, Brussels, Belgium) and golimumab (Centocor, Horsham, Pennsylvania, USA) are the latest tumour necrosis factor (TNF) inhibitors evaluated in double-blind, multicentre randomised controlled trials (RCT). Certolizimab is the pegylated (polyethylene glycolated) Fab′ fragment derived from a high affinity humanised anti-TNFα monocolonal antibody (mAb). Fab′ fragments lack the Fc portion of immunoglobulin, and so the repertoire of Fc-mediated effector responses, such as complement or antibody-dependent cell-mediated cytotoxicity, is distinct from those of other anti-TNF mAb. Nonetheless, it still neutralises membrane TNFα. Derivatisation of the Fab′ fragment prolongs the plasma half-life to 2 weeks. Certolizumab pegol is administered by subcutaneous injection (200 or 400 mg) every 2 weeks. Golimumab is a human anti-TNF mAb administered by subcutaneous injections (50 or 100 mg) every 4 weeks. Three RCT in this issue of Annals of the Rheumatic Diseases ,1–3 (see pages 789, 797 and 805) which report their use in active rheumatoid arthritis (RA), raise two questions. First, do we need more TNF inhibitors? Second, how effective and safe are these new TNF inhibitors? Successful drugs are invariably replicated. “Breakthrough” treatments sound more impressive than subsequent “me too” replications, although the latter products might perform better. Lee4 has elegantly summarised the arguments for “me too” products, indicating that their presence “does not mean that imitation has replaced innovation”. Successful “me too” treatments improve efficacy, reduce toxicity and increase “cost effectiveness”. With biological agents such as TNF inhibitors their added benefits may include more favourable routes or frequencies of administration. There are cogent reasons to anticipate that “me too” biological agents will be commonplace as experience with these agents solidifies and their use increases. There may be special factors with biologicals, particularly around their mechanism of action, which may complicate the comparison of different “me too” products. …
An examination of technical, allocative and economic efficiencies in Ofada rice farming in Ogun State, Nigeria

The need to improve efficiency of rice production is accentuated through high level of importation of rice needs in Nigeria. This paper examined technical, allocative and economic efficiencies in Ofada rice farming in Ogun state, South-West Nigeria and determined factors affecting them among others. A total of 192 rice farmers were sampled through a multi-stage sampling procedure. Data were analyzed using descriptive statistics, stochastic frontier analysis (SFA) and tobit regression analysis. The average areas cultivated to rice was 0.86 ha. Majority (60%) of the farmers had contact with extension workers while 56% had no access to credit. Bird invasion, finance and unavailability of tractors were the most striking problem confronting Ofada rice farming in the opinion of the farmers. The return to scale value of 1.29 estimated from the SFA revealed that farmers were operating in stage I of the production surface, hence, the need to employ more resources in order to maximize benefits. The mean technical, allocative and economic efficiencies were 0.726, 0.928 and 0.674, respectively. It was therefore concluded that rice farmers can still increase output or save cost without the need to change existing technology. Furthermore, extension contact and education were found to be very crucial to efficient rice production. Key words: efficiencies, production, rice, output, return-to-scale, Nigeria.
Venous Thromboembolic Disease in Obstetrics and Gynaecology: The Scottish Experience

The incidence and pattern of thromboembolic complications in obstetric and gynaecological patients in Scotland between 1981 and 1992 was investigated by analysing ICD coded data retrieved from the SMR1 and SMR2 database. The effect of mode of delivery and maternal age over 35 on risk of thromboembolism in pregnancy was also assessed. 0.076% of gynaecological episodes were subsequently complicated by thromboembolic events. Nineteen percent of those suffering thromboembolic complications within two weeks of discharge were referred back to gynaecology. The incidence of deep venous thrombosis (DVT) in those under 35 years and over 35 years was 0.615/1000 maternities and 1.216/1000 maternities respectively. Respective figures for postnatal DVT were 0.304/1000 and O. 720/1000 and for pulmonary thromboembolism (PTE), 0.108/ 1000 and 0.405/1000. In both age groups, emergency caesarean section was associated with a higher incidence of DVT than elective caesarean section and vaginal delivery.
Effect of 1,25(OH)2 vitamin D3 on cytokine production by endometrial cells of women with repeated implantation failure

Repeated implantation failure (RIF) is a worldwide health problem that imposes a great deal of cost on patients and health care system. Vitamin D3 has been proposed to have positive impact on the process of implantation. The present study was performed to compare the effect of 1,25-dihydroxy vitamin D3 (1,25(OH)2D3) on cytokine production by endometrial cells of women with RIF and healthy fertile controls. Whole endometrial cells (WECs) and endometrial stromal cells (ESCs) from RIF and normal fertile women were treated with 1,25(OH)2D3. The levels of IL-10, TGF-β, IFNγ, Il-6, IL-8 and IL-17 in culture supernatants were assayed by ELISA. Also, ability of the cells from both groups to produce 1,25(OH)2D3 was evaluated and compared. 1,25(OH)2D3 down-regulated cytokine production in WECs from both groups except for IL-8 which was upraised. Similar trends were also observed in ESCs except up-regulation of TGF-β in RIF group. Endometrial cells of both groups had comparable capacity to produce 1,25(OH)2D3. Based on the minimal differential immunoregulatory effect of vitamin D3 on endometrial cells from RIF and control women, it may be suggested that circulating levels of maternal vitamin D3 be the subject of further investigation.
Basel requirements of downturn loss given default: modeling and estimating probability of default and loss given default correlations

Basel II requires that banks use downturn loss given default (LGD) estimates in regulatory capital calculations, citing the fact that the probability of default (PD) and LGD correlations are not captured. We show that the lack of correlation can be taken care of by incorporating a certain degree of conservatism in cyclical LGD in a point-in-time (PIT) framework. We examine a model that can capture the PD and LGD correlation in its entirety, differentiating the different components of correlations in question. Using historical LGD and default data of a loan portfolio, we calibrate our model and, through the simulation of economic capital, we show that the mean LGD needs to be increased by about 35% to 41% for a corporate portfolio and about 16% for a mid-market portfolio in order to compensate for the lack of correlations. Our hope is to provide a framework that the banks can use based on their internal data to estimate and justify their LGD choices for different portfolios. Although the paper is presented within the context of Basel II, the applications could be widened to include structured finance, credit derivatives, economic capital and portfolio modeling in general, where PD and LGD correlations need to be estimated and modeled.
Values, prices of production and market prices: some more evidence from the Greek economy

This paper extends the empirical investigation of the relation between labour values and different price forms in the case of the Greek economy. Subjecting the labour theory of value to empirical tests with data from various countries helps in the derivation of general conclusions regarding its empirical validity and practical usefulness. Our results on the closeness of values and prices as measured by their absolute deviation and correlation, the shape of the wage‐profit curves, the predictive power of labour values over market prices compared with other ‘value bases’, and the comparison of fundamental Marxian categories when estimated in value and price terms provide further support for the empirical strength of the labour theory of value.
Environmental Change, Human Resources and Organizational Turnaround

Research on turnaround has largely focused on the impact of retrenchment and repositioning, and has paid less attention to the impact of changes in the task environment and human resources on recovery from decline. Moreover, all of the empirical research on turnaround has been conducted on private organizations. We develop a new model that is derived from theories of environmental and human resource effects on organizational performance. We apply this model to failing school districts in Texas, and find that turnaround is influenced by changes in the munificence and complexity of task environments, and the appointment of a new chief executive and front-line staff.