Miracle-dz/UniFAR-FTUs huggingface dataset:
(CCUG)n RNA toxicity in a Drosophila model of myotonic dystrophy type 2 (DM2) activates apoptosis

ABSTRACT The myotonic dystrophies are prototypic toxic RNA gain-of-function diseases. Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are caused by different unstable, noncoding microsatellite repeat expansions – (CTG)DM1 in DMPK and (CCTG)DM2 in CNBP. Although transcription of mutant repeats into (CUG)DM1 or (CCUG)DM2 appears to be necessary and sufficient to cause disease, their pathomechanisms remain incompletely understood. To study the mechanisms of (CCUG)DM2 toxicity and develop a convenient model for drug screening, we generated a transgenic DM2 model in the fruit fly Drosophila melanogaster with (CCUG)n repeats of variable length (n=16 and 106). Expression of noncoding (CCUG)106, but not (CCUG)16, in muscle and retinal cells led to the formation of ribonuclear foci and mis-splicing of genes implicated in DM pathology. Mis-splicing could be rescued by co-expression of human MBNL1, but not by CUGBP1 (CELF1) complementation. Flies with (CCUG)106 displayed strong disruption of external eye morphology and of the underlying retina. Furthermore, expression of (CCUG)106 in developing retinae caused a strong apoptotic response. Inhibition of apoptosis rescued the retinal disruption in (CCUG)106 flies. Finally, we tested two chemical compounds that have shown therapeutic potential in DM1 models. Whereas treatment of (CCUG)106 flies with pentamidine had no effect, treatment with a PKR inhibitor blocked both the formation of RNA foci and apoptosis in retinae of (CCUG)106 flies. Our data indicate that expression of expanded (CCUG)DM2 repeats is toxic, causing inappropriate cell death in affected fly eyes. Our Drosophila DM2 model might provide a convenient tool for in vivo drug screening. Summary: A Drosophila model of myotonic dystrophy type 2 (DM2) recapitulates several features of the human disease, identifies apoptosis as a contributing factor to DM2, and is likely to provide a convenient tool for drug screening.
The effects of proximal and distal goals on performance on a moderately complex task

The effect of a proximal plus a distal goal was investigated relative to setting only a distal goal or urging participants to do their best. Young adults (N=39) were paid on a piece rate basis to make toys. An analysis of variance revealed that the amount of money earned by the participants who were urged to ‘do your best’ was significantly greater than the amount of money earned by the participants who were assigned a distal goal. However, the amount of money earned by the participants who were assigned proximal goals, in addition to a distal goal, was significantly greater than the amount of money earned by the participants in the ‘do your best’ condition. The correlation between perceived self-efficacy and the amount of money earned was 0.45 (p<0.01). Perceived self-efficacy significantly increased only for those participants in the proximal plus distal goal condition. Proximal goals, through self-efficacy and performance feedback, appear to have focused attention on task appropriate strategies. The results suggest an informational explanation of proximal goals as opposed to a motivational one through goal commitment. Implications of these findings for mentoring and training are discussed. Copyright © 1999 John Wiley & Sons, Ltd.
Sequential processing of “items” and “relations”

Abstract Eichenbaum et al. (1994a) hypothesized that perceptually distinct items and the relations among them are processed sequentially by the parahippocampal region and the hippocampal formation, respectively. Predictions based solely on their model's sequential-processing feature might prove easier to disconfirm than those based on its representational features. Two such predictions are discussed: (1) double dissociations should be impossible following hippocampal vs. parahippocampal lesions, and (2) hippocampal lesions should not exacerbate impairments that follow complete parahippocampal lesions.
Dysregulation of anti-angiogenic agents ( sFlt-1 , PLGF , and sEndoglin ) in preeclampsia — a step forward but not the definitive answer

Preeclampsia (PE) is a pregnancy-specific syndrome characterized by hypertension, proteinuria and edema, which resolves on placental delivery. It is thought to be the consequence of impaired placentation due to inadequate trophoblastic invasion of the maternal spiral arteries. In PE the maternal plasma concentration of free vascular endothelial growth factor (VEGF) and placental growth factor (PLGF) is decreased whereas the concentration of soluble fms-like tyrosine kinase-1 (sFlt-1) and of soluble endoglin (sEng) is increased. These soluble receptors may bind VEGF, PLGF and TGFβl and TGFβ3 in the maternal circulation, causing endothelial dysfunction in many maternal tissues. Hence there is a view that the pathogenesis is more or less clarified. According to the vascular theory, poor placentation leads to poor uteroplacental perfusion and hypoxia, which stimulates sFlt-1 and sEng production causing the maternal syndrome. This assumption has been recently challenged. The role of hypoxia as the main stimulus for release of sFlt-1 has been questioned and the role of inflammatory mechanisms has been emphasized. According to this inflammatory theory, poor placentation may predispose more to placental oxidative stress than hypoxia and endothelial dysfunction may be part of a broader disorder of systemic inflammation. Finally, the recent demonstration of activating auto-antibodies to the angiotensin 1 receptor that experimentally play a major pathogenic role in PE further suggests a pleiotropism of aetiologies for this condition. The purpose of this review is to critically evaluate the recent hypotheses and their possible insights on early diagnosis, prevention and treatment.
Time Perception and the Experience of Time When Immersed in an Altered Sensory Environment

The notion that exposure to a monotonous sensory environment could elicit reports indicating aberrant subjective experience and altered time perception is the impetus for the present report. Research has looked at the influence of exposure to such environments on time perception, reporting that the greater the environmental variation, the shorter is the time estimation obtained by the method of production. Most conditions for creating an altered sensory environment, however, have not facilitated an immersive experience, one that directly impacts both time perception and subjective experience. In this study, we invited our participants to enter a whole-body altered sensory environment for a 20-min session, wherein they were asked to relax without falling asleep. The session included white-colored illumination of the chamber with eyes closed (5 min), followed by 10 min of illuminating the room with color, after which a short report of subjective experience was collected using a brief questionnaire; this was followed by an additional 5 min of immersion in white light with closed eyes. The participants were then interviewed regarding their subjective experience, including their experience of time within the chamber. Prior to entering the chamber, the participants completed a time-production (TP) task. One group of participants then repeated the task within the chamber, at the end of the session; a second group of participants repeated the task after exiting the chamber. We shall report on changes in TP, and present data indicating that when produced time is plotted as a function of target duration, using a log–log plot, the major influence of sensory environment is on the intercept of the psychophysical function. We shall further present data indicating that for those participants reporting a marked change in time experience, such as “the sensation of time disappeared,” their TP data could not be linearized using a log–log plot, hence indicating that for these individuals there might be a “break” in the psychophysical function.
Computer-Mediated Relational Development and Maintenance on Facebook®

This study examines the use of Facebook® for developing and maintaining relationships among 283 college students. Based on theories of relational development and maintenance and research on perceptions of relational partners using computer-mediated communication, the researchers propose a model of relational development/maintenance for Facebook users, and empirically test it using structural equation modeling (SEM). The study focuses on the relationship among relational interdependence, commitment, and predictability. An SEM analysis revealed support for all paths of the model. Implications for computer-mediated relationship maintenance are discussed.
Analysis of motion sensation of car drivers and its application to posture control device

Driver tilts his/her head to the direction of the curve center. Beside, it is pointed out that the head movement of the passenger is opposite to the driver. Moreover, it is known that the driver does not get carsickness comparing with the passenger. Therefore, we investigate the effect of the driver's active head movement. We build the mathematical model of the motion sickness and simulate the severity of motion sickness using driver's head movements measured in the real-car experiments. From the result, it is shown that the driver's head movement has an effect to decrease the motion sickness. Then we proposed a novel posture control device based on the results. It is shown that the developed device changes the passengers head tilt toward the driver's direction, which can decrease the motion sickness.
An aggregator point of view on NL-Means

Patch based methods give some of the best denoising results. Their theoretical performances are still unexplained mathematically. We propose a novel insight of NL-Means based on an aggregation point of view. More precisely, we describe the framework of PAC-Bayesian aggregation, show how it allows to derive some new patch based methods and to characterize their theoretical performances, and present some numerical experiments.
The Role of Integrative Complexity in Military Leadership: Robert E. Lee and His Opponents1

Paragraphs selected from material written by Gen. Robert E. Lee between 1839 and 1867, and by Union generals who opposed him in six major battles, were scored for integrative complexity. This characteristic is defined by the degree of differentiation and integration that the individual demonstrates in processing information, ranging from low or simple (rigid, all-or-none, egocentric) to high or complex (flexible, information-oriented, integrated) on a 1–7 scale. Gen. Lee showed a high level of complexity as a stable characteristic, with changes downward in periods of particular stress. Lee was able to defeat superior enemy forces in battles where his level of complexity was substantially higher than that of the opposing commander; this was not the case when the levels were closer together or when (as with Grant) he faced an opponent whose complexity was actually higher than his own. These findings support previous research on the effects of stress, and emphasize the nature of information processing complexity as both a “trait” and a “state” variable.
AdaptiveSpace-TimeReduced-RankInterference Suppression for Asynchronous DS-CDMA based on a Diversity-Combined Decimation and Interpolation Scheme

An adaptive low-complexity space-time reduced-rank processor is proposed for interference suppression in asynchronous DS-CDMA systems based on a diversity-combined decimation and interpolation method. The novel design approach for the processor employs an iterative procedure to jointly optimize the interpolation, decimation and estimation tasks for reduced-rank parameter estimation. We describe joint iterative least squares (LS) design parameter estimators, propose alternative decimation structures, including the optimal decimation scheme, and develop low complexity adaptive recursive least squares (RLS) algorithms for the proposed structure. Linear space-time receivers with antenna arrays based on the proposed reduced-rank processor are presented and investigated for mitigating multi-access interference (MAI) and intersymbol interference (ISI) in an asynchronous DS-CDMA system uplink scenario.
Early Childhood Teachers' Beliefs about Children's Risky Play in Australia and Norway

Positive risk-taking in the context of outdoor physical play is important for fostering children's optimal health and development. Despite this, there is mounting concern that many developmentally beneficial activities are now seen as dangerous and something to be avoided. However, perceptions of risk are very much subject to cultural interpretation, and the growing risk aversion evident in some developed Western societies, such as Australia, the United Kingdom and the United States of America, is less apparent in other developed countries, notably some of the European and Scandinavian countries. To explore some of these cultural differences, early childhood practitioners from Australia and Norway were interviewed regarding their provision of outdoor play experiences for children and their attitudes towards risk-taking in play. Practitioners from both countries recognised the importance of risky play for children's development and well-being. However, differences in the extent to which children's risky play was supported were evident. Factors associated with the quality of the outdoor environment, regulatory requirements, and a litigious environment were identified as constraining teaching practice for the Australian practitioners. The findings have implications for the development of policy that supports teachers' pedagogical decision-making in providing developmentally challenging play environments for children.
How Much of A “Running Start” Do Dual Enrollment Programs Provide Students?

We study a popular dual enrollment program in Washington State, “Running Start” using a new administrative database that links high school and postsecondary data. Conditional on prior high school performance, we find that students participating in Running Start are more likely to attend any college but less likely to attend four-year colleges in the year after high school graduation. Additionally, we find evidence that data limitations common to previous studies of dual enrollment have substantial impacts on the estimates of dual enrollment on college outcomes.
Maintaining solidarity across generations in New Zealand: Support from mid‐life adult to ageing parent

Abstract This paper establishes the key factors influencing functional solidarity—the material and emotional support mid‐life New Zealanders provide to an ageing parent or in‐law. Using the theoretical framework of intergenerational family solidarity, empirical analysis draws on the 1997 Transactions in the Mid‐Life Family survey of individuals aged 40–54. Results indicate that mature, adult children with no partner and living far from their parent are the least likely to provide emotional and in‐kind help and will be more hesitant to offer emotional support to in‐laws compared to their own parents. Adult children of Christian background, and possibly those who are homemakers and not employed, are more likely to provide in‐kind help, but those in rural areas are less likely. Surprisingly, ill health, a large household, being employed, and continuing to provide help to a young adult child who has left home do not necessarily hinder the respondent in helping an ageing parent. The argument that intergenerational relations are increasingly subject to compromise as mid‐lifers face unprecedented demands for support from other generations needs critical consideration. Further analysis should consider the cultural significance of intergenerational transactions, and how they may be influenced by the cognitive‐affective dimensions of intergenerational family solidarity.
The Ribosome Filter Redux

The ribosome filter hypothesis postulates that ribosomes are not simply translation machines but also function as regulatory elements that differentially affect or filter the translation of particular mRNAs. On the basis of new information, we take the opportunity here to review the ribosome filter hypothesis, suggest specific mechanisms of action, and discuss recent examples from the literature that support it.
Dovepress Open Access to Scientific and Medical Research Open Access Full Text Article Alpha-1-antitrypsin Augmentation Therapy in Deficient Individuals Enrolled in the Alpha-1 Foundation Dna and Tissue Bank

Introduction: Intravenous augmentation therapy with purified intravenous alpha-1 antitrypsin replaces the deficient protein and is the only currently approved treatment for alpha-1 antitrypsin deficiency (AATD) related lung disease. While augmentation therapy has been available for more than 20 years, there are a limited number of studies evaluating the effect of augmentation on lung function. Material and methods: We examined the decline in forced expiratory volume in one second (FEV 1) in patients enrolled in the Alpha-1 Foundation DNA and Tissue Bank in relation to the use or not of alpha-1 antitrypsin augmentation therapy. For the purpose of our analysis we included 164 patients with AATD and PI ZZ genotype. Results: Mean age of the patients was 60 years, 52% were females, 94% were white and 78% ex-smokers. The mean FEV 1 at baseline was 1.7 L and the mean FEV 1 % of predicted was 51.3%. The mean follow-up time was 41.7 months. A total of 124 (76%) patients received augmentation therapy (augmented group) while 40 patients (24%) did not received it (non-augmented group). When adjusted by age at baseline, sex, smoking status, baseline FEV 1 % of predicted, the mean overall change in FEV 1 was 47.6 mL/year, favoring the augmented group (∆FEV 1 10.6 ± 21.4 mL/year) in comparison with the non-augmented group (∆FEV 1 −36.96 ± 12.1 mL/year) (P = 0.05). Beneficial ∆FEV 1 were observed in ex-smokers and the group with initial FEV 1 % of predicted of 50%. No differences were observed in mortality. Conclusions: In conclusion, augmentation therapy improves lung function in subjects with AATD when adjusted by age, gender, smoking status and baseline FEV 1 % of predicted. The beneficial effects were noted in ex-smoker subjects with FEV 1 below 50% of predicted.
Success stories: rhetoric, authenticity, and the right to information movement in north India

This article will look at the use of ‘success stories’ by groups involved in the Right to Information movement in north India. Success stories are narratives tailored by activists to promote active citizenship and to persuade publics that Right to Information legislation can improve accountability in everyday bureaucratic processes by balancing the power relationship between the citizen and the public servant. Success stories might be reproduced as first-hand, text or audio-visual accounts, and the Internet has enabled wide reproduction and dissemination of some narratives in particular. As rhetorical devices, success stories play an important role in activist groups' attempts to frame citizenship and bureaucratic accountability but also have a role in promoting movement cohesion and continuity. Success stories presented at conferences by authentic grassroots activists help to show influential movement sympathisers and policy-makers that the movement is socially diverse and that ordinary people are participating. The conscious use of rhetoric is part of building a sense of belonging to particular groups or to the wider movement. An ethnographic focus on the production, reproduction and performance of success stories reveals the extent to which concern over representation is central to activist practice and identity.
Fairness Expectations and Altruistic Sharing in 15-Month-Old Human Infants

Human cooperation is a key driving force behind the evolutionary success of our hominin lineage. At the proximate level, biologists and social scientists have identified other-regarding preferences – such as fairness based on egalitarian motives, and altruism – as likely candidates for fostering large-scale cooperation. A critical question concerns the ontogenetic origins of these constituents of cooperative behavior, as well as whether they emerge independently or in an interrelated fashion. The answer to this question will shed light on the interdisciplinary debate regarding the significance of such preferences for explaining how humans become such cooperative beings. We investigated 15-month-old infants' sensitivity to fairness, and their altruistic behavior, assessed via infants' reactions to a third-party resource distribution task, and via a sharing task. Our results challenge current models of the development of fairness and altruism in two ways. First, in contrast to past work suggesting that fairness and altruism may not emerge until early to mid-childhood, 15-month-old infants are sensitive to fairness and can engage in altruistic sharing. Second, infants' degree of sensitivity to fairness as a third-party observer was related to whether they shared toys altruistically or selfishly, indicating that moral evaluations and prosocial behavior are heavily interconnected from early in development. Our results present the first evidence that the roots of a basic sense of fairness and altruism can be found in infancy, and that these other-regarding preferences develop in a parallel and interwoven fashion. These findings support arguments for an evolutionary basis – most likely in dialectical manner including both biological and cultural mechanisms – of human egalitarianism given the rapidly developing nature of other-regarding preferences and their role in the evolution of human-specific forms of cooperation. Future work of this kind will help determine to what extent uniquely human sociality and morality depend on other-regarding preferences emerging early in life.
Midge: Generating Descriptions of Images

We demonstrate a novel, robust vision-to-language generation system called Midge. Midge is a prototype system that connects computer vision to syntactic structures with semantic constraints, allowing for the automatic generation of detailed image descriptions. We explain how to connect vision detections to trees in Penn Treebank syntax, which provides the scaffolding necessary to further refine data-driven statistical generation approaches for a variety of end goals.
Judging deservingness and affect in relation to another's employment or unemployment: a test of a justice model

In a study that was based on a structural model of deservingness, 24 male and 80 female undergraduate students responded to scenarios in which either a liked or disliked unemployed stimulus person expended either high or low effort and then either obtained employment or remained unemployed. Results showed that, when the outcome was employment, judgements of responsibility, deservingness, and pleasure about the successful outcome were higher when the stimulus person expended high effort rather than low effort. When the outcome was unemployment, judgements of responsibility, deservingness, and reported pleasure about the unsuccessful outcome were higher when the stimulus person displayed low effort rather than high effort in seeking a job and participants reported feeling less sympathetic and more annoyed with the stimulus person and less inclined to provide financial assistance. Liking relations had most effects in the employed condition. The employed outcome elicited more reported pleasure and less resentment for the liked stimulus person and he or she was judged to be more responsible for and more deserving of employment than the disliked stimulus person. The results were discussed in relation to component balanced and unbalanced structures within the structural model and they also suggested some elaboration of the responsibility variable. © 1998 John Wiley & Sons, Ltd.