Miracle-dz/UniFAR-FTUs huggingface dataset:
Three novel mutations reflect the variety of defects causing phenotypically diverse X‐linked hyper‐IgM syndrome

X‐linked hyper‐IgM syndrome (HIGM1) (MIM♯308230), is a severe primary immunodeficiency caused by mutations in the gene coding for CD40 ligand (CD40L or CD154), a member of the tumour necrosis factor (TNF) superfamily. The interaction of this protein with its ligand, CD40, mediates crucial processes in the immune response. The variety of defects that have been described in HIGM1 patients range from a complete lack of CD40L protein expression to missense mutations that interfere with its interaction with CD40L. In this study we describe three families – a total of seven HIGM1 patients and carriers, presenting a spectrum of severity in clinical evolution. In two of these families, patient DNA samples were available for genetic studies. In the third, carrier detection was performed on female family members. The results of immunological studies – the different patterns of CD40L expression and binding capacity as measured by flow cytometry – and molecular diagnosis are presented. Three novel mutations were identified: an intron mutation that partially interferes with the splicing process (intron 3, position + 5 G/T); a missense mutation (Ser222 Phe) located in the molecular region which interacts with the receptor and which abrogates binding capacity; and a 14 base pair deletion leading to a frameshift and a premature truncated mutation (del I 171 X 195). An attempt to correlate protein expression and function of the CD40L mutants with clinical disease evolution is described.
Recreation conflict among skiers and snowboarders.

Jacob and Schreyer's (1980) four determinants of recreation conflict, along with a measure of perceived safety, were used to examine both out-group and in-group normative beliefs about unacceptable behaviors reported by skiers and snowboarders. Data were obtained from surveys distributed at five ski resorts in Colorado. Bivariate analyses indicated that skiers reported more unacceptable behaviors by snowboarders than by fellow skiers, and snowboarders also identified more out-group than in-group conflict. The skiers and snowboarders in this sample varied in terms of activity style, resource specificity, mode of experience, lifestyle tolerance, and concerns with safety. Two structural equation models were used to identify which determinants influenced beliefs about unacceptable behavior. Only activity style significantly influenced both out-group and in-group conflict in both the skier and the snowboarder path models. The relative influence of the other predictors varied according to activity (skiing or sn.
Accelerated barrier optimization compressed sensing (ABOCS) for CT reconstruction with improved convergence

Recently, we proposed a new algorithm of accelerated barrier optimization compressed sensing (ABOCS) for iterative CT reconstruction. The previous implementation of ABOCS uses gradient projection (GP) with a Barzilai–Borwein (BB) step-size selection scheme (GP-BB) to search for the optimal solution. The algorithm does not converge stably due to its non-monotonic behavior. In this paper, we further improve the convergence of ABOCS using the unknown-parameter Nesterov (UPN) method and investigate the ABOCS reconstruction performance on clinical patient data. Comparison studies are carried out on reconstructions of computer simulation, a physical phantom and a head-and-neck patient. In all of these studies, the ABOCS results using UPN show more stable and faster convergence than those of the GP-BB method and a state-of-the-art Bregman-type method. As shown in the simulation study of the Shepp–Logan phantom, UPN achieves the same image quality as those of GP-BB and the Bregman-type methods, but reduces the iteration numbers by up to 50% and 90%, respectively. In the Catphan©600 phantom study, a high-quality image with relative reconstruction error (RRE) less than 3% compared to the full-view result is obtained using UPN with 17% projections (60 views). In the conventional filtered-backprojection reconstruction, the corresponding RRE is more than 15% on the same projection data. The superior performance of ABOCS with the UPN implementation is further demonstrated on the head-and-neck patient. Using 25% projections (91 views), the proposed method reduces the RRE from 21% as in the filtered backprojection (FBP) results to 7.3%. In conclusion, we propose UPN for ABOCS implementation. As compared to GP-BB and the Bregman-type methods, the new method significantly improves the convergence with higher stability and fewer iterations.
Test Anxiety and its Relationship with Academic Performance among Nursing Students

Introduction: Test anxiety, a common phenomenon among college students, is one of the problems of educational systems. Because test anxiety affects academic performance, this study was performed to determine test anxiety and its relationship with academic performance among nursing students. Methods: In this descriptive- analytical study, 150 students of Abadan Nursing College were selected in 2007. Data were collected via a two-component questionnaire including demographic data and the test anxiety inventory that were completed self-directedly during the final exams. The data were analyzed by SPSS (13.0) software, using some measures of central tendency and variability, Mann Whitney test and Spearman correlation coefficient. Results: The mean score of test anxiety in students was 30.9± 23.2. Fourteen percent of participants did not have any test anxiety, 48.67% had low test anxiety, 34% had moderate test anxiety and 3.33% had severe test anxiety. In sum, 86% had some degrees of test anxiety. In this study, no significant relationship was found between test anxiety and academic performance (r= -0.12, p= 0.13), nor between test anxiety and age (P= 0.84,), nor between test anxiety and marital status (P=0.053), nor between test anxiety and level of education (P=0.057). Conclusion: Regarding the high prevalence of test anxiety in students, far further research is recommended to identify factors relevant to this phenomenon and to present strategies to reduce it.
A constant approximation algorithm for link scheduling in arbitrary networks under physical interference model

Link scheduling is crucial in improving the throughput in wireless networks and it has been widely studied under various interference models. In this paper, we study the link scheduling problem under physical interference model where all senders of the links transmit at a given power <i>P</i> and a link can transmit successfully if and only if the Signal-to-Interference-plus-Noise-Ratio (SINR) at the corresponding receiver is at least a certain threshold. The link scheduling problem is to find a maximum "independent set" (MIS) of links, <i>i.e.</i>, the maximum number of links that can transmit successfully in one time-slot, given a set of input links. This problem has been shown to be NP-hard [10]. Here we propose the first link scheduling algorithm with a constant approximation ratio for arbitrary background noise <i>N</i> ≥ <i>0</i>. When each link <i>l</i> has a weight <i>w</i>(<i>l</i>)><i>0</i>, we propose a method for weighted MIS with approximation ratio <i>O</i>(min(log max<sub>ι∈<i>L</i></sub> <i>w</i>(ι)/min<sub>ι∈<i>L</i></sub> <i>w</i>||ι||, log max<sub>ι∈<i>L</i></sub> <i>w</i>(ι)/min<sub>ι∈<i>L</i></sub> <i>w</i>||ι||, where ||ι|| is the Euclidean length of a link ι.
Cognitive Dysfunction in Systemic Lupus Erythematosus: Prevalence and Correlates

Little is known about the mechanisms and relevance of cognitive dysfunction in systemic lupus erythematosus (SLE) patients who never displayed major neuropsychiatric manifestations (nSLE). Thirty-one nSLE female patients and 31 cognitively healthy control women were recruited. Sociodemographic, clinical, neuropsychological and SLE-related markers were collected including cerebral perfusion by single-photon emission computed tomography. Prevalences of cognitive complaints were 22.6% in nSLE versus 6.5% in the control group (p = 0.147); respective prevalences of cognitive dysfunction were 32.3 versus 6.5% (p = 0.01). Within the nSLE group, all cognitive domains appeared similarly affected, and correlations were found between cognitive dysfunction and less skilled occupation (r = –0.41, p = 0.02) and between cognitive complaints and depressive symptoms (r = 0.35, p = 0.05). Cognitive dysfunction is rather frequent in nSLE and seems to negatively impinge on social functioning.
The Association between Neuroticism and Heart Rate Variability Is Not Fully Explained by Cardiovascular Disease and Depression

Neuroticism is associated with cardiovascular disease, autonomic reactivity, and depression. Here we address the extent to which neuroticism accounts for the excess heart disease risk associated with depression and test whether cardiac autonomic tone plays a role as mediator. Subjects were derived from a nationally representative sample (n = 1,255: mean age 54.5, SD = 11.5). Higher neuroticism was associated with reduced heart rate variability equally under rest and stress. The baseline structural equation model revealed significant paths from neuroticism to heart rate variability, cardiovascular disease and depression, and between depression and cardiovascular disease, controlling for age, sex, height, weight, and BMI. Dropping both the neuroticism to heart rate variability, and neuroticism to heart disease paths significantly reduced the model fit (p < .001 in each case). We conclude that neuroticism has independent associations with both autonomic reactivity and cardiovascular disease, over and above its associations with depression and other related variables.
Understanding CNS remyelination: Clues from developmental and regeneration biology

A guiding principle in remyelination research has been to seek clues to its nature in developmental studies on myelination. This “recapitulation hypothesis” argues that the regenerative response involves rerunning much the same programme as occurs during the developmental process. Here we examine the extent to which current evidence supports this hypothesis and whether this is a useful conceptual framework within which to study remyelination and suggest that an equally fruitful approach is to look to regenerative processes in other tissues. J. Neurosci. Res. 58:207–213, 1999. © 1999 Wiley‐Liss, Inc.
Impact of implant support for mandibular dentures on satisfaction, oral and general health-related quality of life: a meta-analysis of randomized-controlled trials.

OBJECTIVES The aim of this study was to examine systematically the data published on the efficacy of mandibular implant-retained overdentures from the patient's perspective. MATERIAL AND METHODS Medline, Embase, The Cochrane Central Register of Controlled Trials and The Cochrane Systematic Reviews Database were searched and complemented by hand searching. All randomized-controlled trials published in English or French up to April 2007 were included, in which conventional dentures and mandibular implant overdentures in adult edentulous individuals were compared. The outcomes of interest were patient satisfaction, oral and general health-related quality of life. Random effects models were used to pool the effect sizes (ES) of all included studies. RESULTS Ten publications of seven randomized-controlled trials were identified and eight were included in the meta-analysis. When compared with mandibular conventional dentures, implant overdentures were rated to be more satisfactory at a clinically relevant level [pooled ES 0.80, z=3.56, 95% confidence intervals (CI) 0.36-1.24, P=0.0004], but a statistical heterogeneity was found (chi(2)=31.63, df=5, P<0.00001, I(2)=84%). The pooled ES for oral health quality of life was -0.41 (z=1.31, 95% CI, -1.02 to 0.20; P=0.19, chi(2)=11.53, df=2, P<0.003, I(2)=83%). There was a lack of evidence to show the impact of mandibular implant overdenture on perceived general health. CONCLUSIONS Our findings suggest that, although mandibular implant-retained overdentures may be more satisfying for edentulous patients than new conventional dentures, the magnitude of the effect is still uncertain. There is a need for additional evidence including cost-effectiveness analyses on the impact of mandibular implant overdentures and conventional dentures.
Zimbabweans in Britain: Transnational Activities and Capabilities

This paper explores the economic, political, social and cultural transnational exchanges of 500 Zimbabweans living in the UK. The paper examines the ways in which motivations for migration and immigration status impact on the type of transnational exchanges that take place between the UK and Zimbabwe, and also among diasporic networks elsewhere. After social contact with close family members, economic remittances form the most regular exchanges. The paper shows the ways in which remittance activities are affected by the structural exclusions, due to government policy, experienced by asylum-seekers and undocumented migrants who are prohibited from working in the regular labour market. However, it also clearly demonstrates that, with the exception of more political activity among those who left Zimbabwe for political reasons, motivations for migration have little impact on the nature of transnational exchanges among Zimbabweans in the UK.
Engaging in Work Even When It Is Meaningless

The central aim of the present study was to assess the predictive value of affective disposition and meaningful work on employee engagement. Specifically, it was proposed that meaningful work moderates the relationship between affective disposition and engagement. Questionnaires were completed by 252 white-collar employees, working in a variety of organizations and jobs across Israel, recruited from community-based samples on a voluntary basis. As hypothesized, work engagement, affective disposition, and meaningful work were positively correlated. Additionally, a significant interaction between affective disposition and meaningful work was found. The relationship between affective disposition and work engagement was found to differ by the extent to which individuals perceived their work as meaningful. Specifically, when work was not perceived as meaningful, employees characterized by high scores on affective disposition were more strongly engaged compared to employees who were characterized by low scores on affective disposition. However, when work was perceived as meaningful, there was no difference in level of engagement found between those with high or low scores on affective disposition. The implications of these results are discussed.
Advances in Medical Education and Practice Dovepress Evaluation of the Flipped Classroom Approach in a Veterinary Professional Skills Course

Background: The flipped classroom is an educational approach that has had much recent coverage in the literature. Relatively few studies, however, use objective assessment of student performance to measure the impact of the flipped classroom on learning. The purpose of this study was to evaluate the use of a flipped classroom approach within a medical education setting to the first two levels of Kirkpatrick and Kirkpatrick's effectiveness of training framework. Methods: This study examined the use of a flipped classroom approach within a professional skills course offered to postgraduate veterinary students. A questionnaire was administered to two cohorts of students: those who had completed a traditional, lecture-based version of the course (Introduction to Veterinary Medicine [IVM]) and those who had completed a flipped classroom version (Veterinary Professional Foundations I [VPF I]). The academic performance of students within both cohorts was assessed using a set of multiple-choice items (n=24) nested within a written examination. Data obtained from the questionnaire were analyzed using Cronbach's alpha, Kruskal–Wallis tests, and factor analysis. Data obtained from student performance in the written examination were analyzed using the nonparametric Wilcoxon rank sum test. Results: A total of 133 IVM students and 64 VPF I students (n=197) agreed to take part in the study. Overall, study participants favored the flipped classroom approach over the traditional classroom approach. With respect to student academic performance, the traditional classroom students outperformed the flipped classroom students on a series of multiple-choice items (IVM mean =21. Conclusion: This study demonstrates that learners seem to prefer a flipped classroom approach. The flipped classroom was rated more positively than the traditional classroom on many different characteristics. This preference, however, did not translate into improved student performance, as assessed by a series of multiple-choice items delivered during a written examination.
Child Rearing and Compliance

The relation between parental child-rearing practices and child compliance was examined in two groups of middle- to upper-middle-class parents in Houston, Texas: 20 Japanese families temporarily living in the city and 18 Caucasian American families. The children were 4- to 7-year-olds enrolled in summer sessions in a private preschool/elementary school. Mothers and fathers were interviewed separately about their child-rearing practices, and parents and teachers completed child behavior questionnaires. In both Japanese and American families, child compliance with adult authority was positively associated with providing opportunities for appropriate behavior and negatively associated with reliance on punishment and physical intervention. Japanese parents relied less than Americans on external punishments and more on verbal techniques alone. American parents were more likely to provide opportunities and to use praise. For Japanese families, the length of exposure to American culture was associated with predictable shifts in parental child-rearing practices and child behavior.
The Challenge of Unattended Children in the Public Library.

Public libraries will continue to be challenged by the presence of unattended children in the library as long as parents struggle to provide adequate childcare arrangements. This article discusses some typical library responses to the presence of unattended children and identifies elements that are shared by the most successful programs. Many libraries have found that offering programs for children is an excellent way to strengthen the relationship between the library and its community. In addition, innovative programs that encourage young library users provide an opportunity to foster a new generation of library users, which is critical if libraries are to survive as an institution in the coming years.
What comes after compulsory education? A follow‐up study on parental expectations of their child’s future education

This paper examines the contribution of parents’ education and children’s gender on parental expectations of their children’s future education and the role of parental perceptions of their child’s competencies in the formation of their expectations. A group of university and vocationally educated parents (N = 418) were asked to estimate the probability of their child entering gymnasium (high school) or vocational education and assess the child’s competencies, first in preschool, and then at the end of the third school year. It was found that the education and gender‐bound differences in the parental expectations were established before the child entered school, and by the end of the third school year the relationships between expectations and competence assessments strengthened and were more uniform among the parents. The findings suggested that the parental assessments of their child’s abilities can be regarded as a potentially important social‐psychological process through which social differences are transformed into the individualized interpretations of the child’s educational prospects.
Too little and too much trust: performance measurement in Australian higher education

A striking feature of contemporary Australian higher education governance is the strong emphasis on centralized, template style, metric-based, and consequential forms of performance measurement. Such emphasis is indicative of a low degree of political trust among the central authorities in Australia in the intrinsic capacity of universities and academics to do their work efficiently and effectively. At the same time, it is indicative of a deep-seated political trust in highly centralized and top–down forms of performance measurement, and their capacity to effectively control and coordinate the work of universities and academics. In this article, we argue with regard to performance measurement that these patterns of trust and mistrust embody contradictory assumptions regarding the agency and motivations of universities and academics and prevent adequately coming to terms with the unintended effects that the current performance measurement regime has on universities and the academics working in them.
Overconfidence is universal ? Depends what you mean

1 Department of Human Evolutionary Biology, Harvard University, 11 Divinity Avenue, Cambridge, MA 02138 USA 2 Department of Social Psychology, London School of Economics and Political Science, 55/56 Lincoln’s Inn Fields, London WC2A 3LJ UK 3 Department of Psychology, University of British Columbia, 2136 West Mall, Vancouver V6T 1N4 Canada 4 Department of Behavioral Science, Hokkaido University N10W7, Kita-ku, Sapporo, Hokkaido, 060-0810 Japan 5 Japan Society for the Promotion of Science, 8 banchi, 1 ban-cho, Chiyoda-ku, Tokyo, 102-8472 Japan 6 Department of Psychology, The Chinese University of Hong Kong, Shatin, NT, Hong Kong 7 Department of Social Psychology, The University of Tokyo, 7-3-1 Bunkyo-ku, Tokyo, 1130033 Japan 8 Center for Experimental Research in Social Sciences, Hokkaido University, N10W7, Kita-ku, Sapporo, Hokkaido, 060-0810 Japan 9 Canadian Institute for Advanced Research
Galanin inhibits GLP‐1 and GIP secretion via the GAL1 receptor in enteroendocrine L and K cells

Galanin is a widely expressed neuropeptide, which in the gut is thought to modulate gastrointestinal motility and secretion. We aimed to elucidate the poorly characterised mechanisms underlying the inhibitory effect of galanin and the potential involvement of G‐protein coupled inwardly rectifying potassium, Kir3, (GIRK) channels in glucagon‐like peptide 1 (GLP‐1) and glucose‐dependent insulinotropic polypeptide (GIP) secretion.
A mutant T7 RNA polymerase as a DNA polymerase.

We have identified a T7 RNA polymerase (RNAP) mutant that efficiently utilizes deoxyribonucleoside triphosphates. In vitro this mutant will synthesize RNA, DNA or ‘transcripts’ of mixed dNMP/rNMP composition depending on the mix of NTPs present in the synthesis reaction. The mutation is conservative, changes Tyr639 within the active site to phenylalanine and does not affect promoter specificity or overall activity. Non‐conservative mutations of this tyrosine also reduce discrimination between deoxyribo‐ and ribonucleoside triphosphates, but these mutations also cause large activity reductions. Of 26 mutations of other residues in and around the active site examined none showed marked effects on rNTP/dNTP discrimination. Mutations of the corresponding tyrosine in DNA polymerase (DNAP) I increase miscoding, though effects on dNTP/rNTP discrimination for the DNAP I mutations have not been reported. This conserved tyrosine may therefore play a similar role in many polymerases by sensing incorrect geometry in the structure of the substrate/template/product due to inappropriate substrate structure or mismatches. T7 RNAP can use RNA templates as well as DNA templates and is capable of both primer extension and de novo initiation. The Y639F mutant retains the ability to use RNA or DNA templates. Thus this mutant can display de novo initiated or primed DNA‐directed DNA polymerase, reverse transcriptase, RNA‐directed RNA polymerase or DNA‐directed RNA polymerase activities depending simply on the templates and substrates presented to it in the synthesis reaction.