Miracle-dz/UniFAR-FTUs huggingface dataset:
Disease onset in X-linked dystonia-parkinsonism correlates with expansion of a hexameric repeat within an SVA retrotransposon in TAF1

Significance The genetic basis of X-Linked dystonia-parkinsonism (XDP) has been difficult to unravel, in part because all patients inherit the same haplotype of seven sequence variants, none of which has ever been identified in control individuals. This study revealed that one of the haplotype markers, a retrotransposon insertion within an intron of TAF1, has a variable number of hexameric repeats among affected individuals with an increase in repeat number strongly correlated with earlier age at disease onset. These data support a contributing role for this sequence in disease pathogenesis while further suggesting that XDP may be part of a growing list of neurodegenerative disorders associated with unstable repeat expansions. X-linked dystonia-parkinsonism (XDP) is a neurodegenerative disease associated with an antisense insertion of a SINE-VNTR-Alu (SVA)-type retrotransposon within an intron of TAF1. This unique insertion coincides with six additional noncoding sequence changes in TAF1, the gene that encodes TATA-binding protein–associated factor-1, which appear to be inherited together as an identical haplotype in all reported cases. Here we examined the sequence of this SVA in XDP patients (n = 140) and detected polymorphic variation in the length of a hexanucleotide repeat domain, (CCCTCT)n. The number of repeats in these cases ranged from 35 to 52 and showed a highly significant inverse correlation with age at disease onset. Because other SVAs exhibit intrinsic promoter activity that depends in part on the hexameric domain, we assayed the transcriptional regulatory effects of varying hexameric lengths found in the unique XDP SVA retrotransposon using luciferase reporter constructs. When inserted sense or antisense to the luciferase reading frame, the XDP variants repressed or enhanced transcription, respectively, to an extent that appeared to vary with length of the hexamer. Further in silico analysis of this SVA sequence revealed multiple motifs predicted to form G-quadruplexes, with the greatest potential detected for the hexameric repeat domain. These data directly link sequence variation within the XDP-specific SVA sequence to phenotypic variability in clinical disease manifestation and provide insight into potential mechanisms by which this intronic retroelement may induce transcriptional interference in TAF1 expression.
Value, Size, Momentum, and Unique Role of Microcaps in CEE Market Stock Returns

This article examines cross-sectional asset-pricing methods based on value, size, and momentum on the Central and Eastern European (CEE) markets. It highlights the unique role of microcap equities. The analysis is based on stock-level data for 11 countries within the period from April 2001 to June 2014. It provides strong evidence for the value and momentum effects, simultaneously questioning the traditional size premium. Furthermore, the study indicates exceptionally high returns on microcaps. Except for the momentum and market risk premium, it reveals no correlation between local CEE pricing factors and global, or Western European, factors. Although both three-factor and four-factor models based on local factors outperform the models based on international factors, they fail to explain the abnormal returns observed in the case of microcaps. However, the introduction of a new pricing factor—micro minus rest (MMR)—leads into nearly entire rectification of this defect.
Economic factors, firm characteristics and performance: a panel data analysis for United Kingdom life offices

This article examines panel data evidence to investigate the linkage between insurer performance proxied by investment yield and several economic and firm-specific factors in the United Kingdom life insurance industry. The empirical results indicate that investment yield is positively related interest rate level, but negatively related to interest rate changes, reinsurance dependence, assets held to cover linked liabilities and instability of asset structure.
Outlyingness Weighted Covariation

Quadratic covariation is a popular descriptive measure for the volatility of a multivariate price process. It is consistently estimated by the sum of outer products of high-frequency returns. The proposed realized outlyingness weighted covariation (ROWCov) is a weighted sum of outer products of high-frequency returns and downweights returns that, because of jumps or other reasons, are outliers under the Brownian semimartingale model. The ROWCov is positive semidefinite and remains consistent for the integrated covariance in the presence of a finite-activity jump process. We illustrate the usefulness of the estimator on five-minute returns on the transaction prices of the Dow Jones Industrial Average constituents.
A Model of Pedestrian Route Choice and Demand for Retail Facilities within Inner-City Shopping Areas

There are still only a few operational models of pedestrian movement. In particular, the gravity /entropy-maximizing model has received most attention. In this paper a descriptive model of pedestrian movement is presented. It can be considered as an extension of O'Kelly's model of the demand for retail facilities in the presence of multistop, multipurpose trips. The model basically consists of three submodels: one for destination choice, one for route choice, and one for impulse stops. Together, these submodels describe/predict the total demand for retail facilities within inner-city shopping areas. The model is applied to data from the city of Maastricht, The Netherlands.
Socially Responsible Investment Fund Performance: The Impact of Screening Intensity

Perhaps the most common criticism of socially responsible investment funds is that imposing non-financial screens restricts investment opportunities, reduces diversification efficiencies and thereby adversely impacts performance. In this study we investigate this proposition and test whether the number of screens employed has a linear or curvilinear relation with return. Moreover, we analyse the link between screening intensity and risk. Screening intensity has no effect on unadjusted (raw) returns or idiosyncratic risk. However, we find a significant reduction in α of 70 basis points per screen using the Carhart performance model. Increased screening results in lower systematic risk – in line with managers choosing lower β stocks to minimize overall risk.
The changing business model of B‐schools

Purpose – The purpose of this paper is to argue that recent changes in the wider contexts of universities and business schools signal a shift in their business model instead of a mere turn in their business cycle.Design/methodology/approach – The paper reviews the position of those that hold a traditionalist view of the business model of business schools and comments on the extent to which such a model applies to the ever‐changing conditions that are a feature of the environment of institutions of higher learning.Findings – The paper argues that the process of globalization augments the effects of five sector‐specific forces on the business model of business schools. Additionally, the paper identifies some structural factors that business schools might wish to address in order to cope with changes occurring in their wider environments.Originality/value – In addition to structural measures, the paper proposes a number of specific actions that business schools may implement to maintain and/or gain competiti.
Psychological contract framework on the linkage between developmental human resource configuration and role behavior

The purpose of this study was to examine the role of psychological contract on the linkage between developmental human resource (HR) configurations and role behavior relationships. Multilevel analysis of dyad data revealed that developmental HR configurations could strengthen employees' organizational behaviors that benefit the organization (OCBO) in general by simultaneously shaping their relational and balanced psychological contracts. Further, developmental HR configurations would also improve employees' organizational behaviors that benefit specific individuals (OCBI) and indirectly contribute to the organization by fostering their relational psychological contracts. These findings have implications for research on HR management and psychological contracts as well as for managers seeking to enhance or maintain a firm's competitive advantages.
Dubious Sovereignty: Federal Conditions of Aid and the No Child Left Behind Act

The No Child Left Behind Act (NCLB) represents the greatest extension to date of Federal authority over public school governance. In NCLB, Congress used its conditional spending power to push states and localities into enacting particular kinds of testing and accountability policies. This article places NCLB in the context of Congress's generally increasing willingness to exert itself via conditions attached to federal financial aid. It also analyzes the implications of NCLB for federalism and intergovernmental relationships in education governance.
Sensitivity associated with the identification of visceral adipose tissue levels using waist circumference in men and women: effects of weight loss.

OBJECTIVE The first objective was to determine the associated sensitivity (true positives) and specificity (false positives) associated with the use of waist circumference (WC) to predict specific visceral adipose tissue (VAT) levels. Because WC is a strong correlate of VAT, and, a WC of 100 cm and a VAT area of 130 cm2 (L4-L5) have been suggested as thresholds for increased disturbances in insulin-glucose homeostasis and lipid metabolism, we assessed the sensitivity and specificity when using a WC of 100 cm to identify a VAT of 130 cm2 or greater. A second objective was to evaluate the effects of diet and exercise induced weight loss on VAT and WC to establish the utility of using WC to follow reductions in VAT. RESEARCH DESIGN AND METHODS The sensitivity between WC and VAT was assessed using 50 male (BMI: 30.9 +/- 4.7, age: 41.0 +/- 11.2 y) and 49 female (BMI: 33.3 +/- 5.0, age: 36.9 +/- 7.4 y) subjects. The effects of weight loss on WC and VAT were evaluated in a subgroup of 20 male and 38 female subjects. VAT was measured by magnetic resonance imaging. RESULTS The sensitivity associated with the prediction of a VAT of 130 cm2 or greater using a WC of 100 cm was high (83%), however, so too was the incidence of false positives (38%). Significant (p < 0.01) correlations were obtained between concurrent reductions in VAT and WC for both the male (r = 0.69) and female (r = 0.47) groups. For the two groups combined, regression analysis indicated that a 1 cm reduction in WC was associated with a 4% reduction in VAT mass (p < 0.01). CONCLUSIONS The misclassification of individuals with VAT accumulation above 130 cm2 suggest that WC alone lacks the sensitivity required to identify specific VAT values. The diet and exercise induced reductions in visceral adiposity are positively related to corresponding reductions in WC for both genders is a positive result, and demonstrates the potential utility of using WC to follow changes in the AT depot that conveys the greatest health risk.
Cutaneous pigmented stripes and bleomycin treatment.

To the Editor. — Bleomycin sulfate is a cytostatic antibiotic that is extensively used in cancer therapy. It is often included in protocols of polychemotherapy with cisdiamino-platinum for the treatment of testicular dysembryomas and head and neck tumors. Among the frequent side effects of bleomycin is bandlike cutaneous pigmentation. In a series of 15 patients treated with polychemotherapy, including bleomycin sulfate (effective dose, 160 mg), we noted that ten of them developed cutaneous pigmented stripes. Such skin lesions are classic, although there was no pruritus noted in our patients, contrary to data presented in the literature. There were no other side effects on skin, mucous membrane, hair, or nails, nor were there any visceral lesions (eg, in the lung). These cutaneous lesions were isolated. There was no pigmentation of the palmar flexure lines as well as none of the other commonly described lesions such as tender sclerodermalike infiltration of the extremities,
Agenda Setting and the Blogosphere: An Analysis of the Relationship between Mainstream Media and Political Blogs

This paper asks: what is the relationship between the mainstream media and blog agendas? To be more precise, this paper tracks media coverage and blog discussion of 35 issues during the 2004 presidential campaign to test the hypothesis that the mainstream media agenda exerts a substantial impact on the blog agenda against the increasingly popular hypothesis that the blog agenda exerts a strong influence on the mainstream media agenda. Using a computer-assisted, quantitative content analysis of ten randomly selected A-list political blogs and 50 randomly selected, less popular political blogs over the five-month period from July 1 to November 30, 2004, the author finds that on the vast majority of issues there was a complex, bidirectional relationship between mainstream media coverage and blog discussion rather than a unidirectional media or blog agenda-setting effect. Copyright 2007 by The Policy Studies Organization.
VISUAL RESOLUTION IN HUMANS FLUCTUATES OVER THE 24H PERIOD*

The present experiment was designed to assess daily fluctuations of visual discriminability, a function reflecting the resolution power of the visual sensitivity by measure of a differential threshold. Sixteen subjects underwent a visual discrimination threshold task (using the constant method) in a protocol allowing one point every 2h over the 24h period. The results show that the visual discrimination threshold is low in the morning and increases progressively over the day, reaching a first peak at 22:00. During the night, the same pattern occurs, with low threshold levels at the beginning of the night and high levels at the end. This profile is quite different from that of detection threshold variations, suggesting that the two visual functions are under the control of different underlying mechanisms. Two interpretations could account for this discrepancy. The first relates to different oscillators in the eye for detection and discrimination. The second refers to a possible linkage of visual discriminability with the sleep-wake cycle since threshold measures were systematically low (i.e., high resolution power) after long sleep periods. (Chronobiology International, 17(12), 187–195, 2000)
Differentiation of choroid plexus ependymal cells into astrocytes after grafting into the pre‐lesioned spinal cord in mice

Choroid plexus epithelial cells represent a continuation of, and have the same origin as, ventricular ependymal cells, and are regarded as modified ependymal cells. To extend previous studies of the use of choroid plexus ependymal cell (CPEC) grafting for nerve regeneration in the spinal cord, we investigated the capacity of cultured choroid plexus ependymal cells to differentiate into other types of glial cells in the spinal cord tissue. The choroid plexuses were excised from the fourth ventricle of green fluorescent protein (GFP)‐transgenic mice and the cells were dissociated and cultured for 4–6 weeks. CPECs were harvested from the monolayer cultures and injected into the pre‐lesioned spinal cords of wild‐type mice of the same strain using a Hamilton syringe. One week after injection, some GFP‐positive transplanted cells became immunohistochemically positive for glial fibrillary acidic protein (GFAP) but negative for neurofilament and myelin basic protein. All the GFAP‐positive transplanted cells were negative for vimentin. Two weeks after grafting, immunoelectron microscopy showed that the GFP‐positive transplanted cells that had gained GFAP immunoreactivity contained numerous bundles of intermediate filaments, a morphological characteristic similar to that of astrocytes, and were in close contact with adjacent host tissue. These results indicate that, when grafted into the spinal cord, at least some cultured choroid plexus ependymal cells have the capacity to differentiate into astrocytes. GLIA 36:364–374, 2001. © 2001 Wiley‐Liss, Inc.
Inside the “black box”: the performance of boards of directors of unlisted companies

Purpose – The purpose of this paper is to explore two under‐developed areas of board research: the corporate governance of unlisted companies; and board behaviour, focusing on process factors that contribute to a board performing effectively.Design/methodology/approach – The Board Effectiveness Questionnaire was completed by 67 directors, to gather views on how their board currently operates and how they think it should operate, across various behavioural areas relating to the relationships, decision making, the working climate and predispositions. Analysis of “process losses”, the pattern of sub‐optimal board behaviour, allowed the testing of four hypotheses concerning board effectiveness.Findings – The 18 most important and five least important behaviours were identified, plus 12 showing the greatest “process loss”. Quality of team “relationships” is the main cause of loss whereas evidence for the impact of leadership style is mixed. Size of the board is related to overall performance of the board but n.
Tolls, Exchange Rates, and Borderplex International Bridge Traffic

Budget constraints are forcing many governments to consider implementing tolls as a means for financing bridge and road expenditures. Newly available time series data make it possible to analyze the impacts of toll variations and international business cycle fluctuations on cross-border bridge traffic between El Paso and Ciudad Juarez. Parameter estimation is carried out using a linear transfer function ARIMA methodology. Price elasticities of demand are similar to those reported for other regional economies, but out-of-sample forecasting results are mixed.
Operational risk in Islamic banks: examination of issues

Purpose - The purpose of this paper is to assess key issues in measurement and management of operational risk in Malaysian Islamic banks. Design/methodology/approach - Descriptive, analytical, and comparative analyses are used to discuss the issues of operational risk in Islamic bank through the implications associated with the Islamic banks' operational risk as well as the implications on risk measurement, risk management, and capital adequacy. Findings - Discussion on operational risk in Islamic banks is significant and becoming more complicated compared with conventional banking because of the unique contractual features and general legal environment. While basic Basel II core principles of effective banking supervision apply equally well and ideally suit the Islamic banking institutions, risk measurement, and risk management practices still need specific adaptations to Islamic banks' operational characteristics. These particularities highlight the unique characteristics of Islamic banks and raise serious concerns regarding the applicability of the Basel II methodology for Islamic banks. Research limitations/implications - This study has important implications for the understanding of operational risk, particularly the specific issues of the Islamic banks' operational risk that arise from the different nature of the financing and investment activities of the banks. With regard to measuring operational risk capital charge, the banks have to choose the right and effective method to ensure the operational risk capital charge will be more in line with the banks' actual risk profile and thus will provide the adequate capital and an improved buffer once the losses are announced. Originality/value - The paper will fill the gap to the existing literature of operational risk in banking institutions especially Islamic banks, by showing the needs of specific adaption of operational risk measurement and risk management practices due to the nature of Islamic banks.
Tracking Local Unions Involved in Managerial Decision-Making

This paper reviews the empirical literature on the impact of union and worker participation in managerial decision-making on the union as an institution. It then looks at the experience over time of eleven local unions heavily involved in various forms of participation, trac ing both the trajectory of participation and union outcomes. The outcomes examined include membership levels, member participa tion, leadership development, internal politics, collective bargaining and grievance handling impacts, and enterprise unionism. Analyses of these cases suggest that participation may be declining. At the same time, participation does not seem to have profoundly altered the nature and functioning of these unions. Further, participation does not appear to "cause" enterprise unionism, but local unions that are isolated economically and politically do have trouble coping with participation. Finally, overall, these local unions often lack resources to do both participation and their traditional functions well.
The Albanians of Kosovo and Macedonia: Ethnic identity superseding religion

The Albanians are divided between three religions: the Catholics, the Orthodox, and the Muslims. The religious groups participated in the national development of the Albanians around 1900 each in its own way and in proportion to its own strength. As the majority group, the Muslims (70–80%) played the most important role. Most of the Muslims wanted to remain within the Ottoman Empire as long as their traditional rights were maintained, in spite of a strong desire for local self-government, but, as the Ottoman Empire declined, radical Albanian leaders stressing cultural and linguistic unity rather than religious unity were to gain the upper hand. The Albanian language, culture, and feeling of common blood played the most important roles in Albanian nationalism. The Albanian language in particular gave Albanians the feeling of belonging to the same nation.
Long–Term Orientation and Intertemporal Choice in Family Firms

A long–term orientation (LTO) is often associated with family firms, but the LTO construct is underdeveloped. This paper sets forth a framework for studying LTO in family firms including developing three dimensions—futurity, continuity, and perseverance. It identifies LTO as a higher–order heuristic that, in matters of intertemporal choice, provides a dominant logic for decisions and actions. Intertemporal choice refers to decisions with payoffs or outcomes that play out over time. Three mechanisms affecting intertemporal choices are identified—representation, self–control, and anticipation. LTO and intertemporal choice are further examined and discussed in the context of family firms.
Left Hypofrontality Correlates with Blunted Affect in Schizophrenia

Abstract: Regional cerebral blood flow (rCBF) during the resting state was measured using the 133Xe inhalation technique in 39: patients diagnosed as having schizophrenic disorder according to the DSM‐III criteria and 30: age‐ and sex‐matched healthy volunteers. All the patients were receiving neuroleptic medication. The bilateral hemispheric mean flow values in the schizophrenic patients decreased significantly and the relative flow distribution reduced significantly only in the left frontal region compared to the controls. The lower the left frontal blood flow in schizophrenics, the more pronounced were the negative symptoms including blunted aflect, avolition‐apathy and inattention. These results suggest that the negative symptoms in schizophrenics are related to left frontal lobe dysfunction.