bluuebunny/crossref_metadata_2025_split huggingface dataset:
Differential phenotypic expression of a novel PDHA1 mutation in a female monozygotic twin pair
Authors: Horga Alejandro , Woodward Catherine E. , Mills Alberto , Pareés Isabel , Hargreaves Iain P. , Brown Ruth M. , Bugiardini Enrico , Brooks Tony , Manole Andreea , Remzova Elena , Rahman Shamima , Reilly Mary M. , Houlden Henry , Sweeney Mary G. , Brown Garry K. , Polke James M. , Gago Federico , Parton Matthew J. , Pitceathly Robert D. S. , Hanna Michael G. 
Published: October 2019

Abstract
Pyruvate dehydrogenase complex (PDC) deficiency caused by mutations in the X-linked PDHA1 gene has a broad clinical presentation, and the pattern of X-chromosome inactivation has been proposed as a major factor contributing to its variable expressivity in heterozygous females. Here, we report the first set of monozygotic twin females with PDC deficiency, caused by a novel, de novo heterozygous missense mutation in exon 11 of PDHA1 (NM_000284.3: c.1100A>T). Both twins presented in infancy with a similar clinical phenotype including developmental delay, episodes of hypotonia or encephalopathy, epilepsy, and slowly progressive motor impairment due to pyramidal, extrapyramidal, and cerebellar involvement. However, they exhibited clear differences in disease severity that correlated well with residual PDC activities (approximately 60% and 20% of mean control values, respectively) and levels of immunoreactive E1α subunit in cultured skin fibroblasts. To address whether the observed clinical and biochemical differences could be explained by the pattern of X-chromosome inactivation, we undertook an androgen receptor assay in peripheral blood. In the less severely affected twin, a significant bias in the relative activity of the two X chromosomes with a ratio of approximately 75:25 was detected, while the ratio was close to 50:50 in the other twin. Although it may be difficult to extrapolate these results to other tissues, our observation provides further support to the hypothesis that the pattern of X-chromosome inactivation may influence the phenotypic expression of the same mutation in heterozygous females and broadens the clinical and genetic spectrum of PDC deficiency.
Ultraviolet radiation modulates both constitutive and inducible plant defenses against thrips but is dose and plant genotype dependent
Authors: Escobar-Bravo Rocío , Nederpel Charlotte , Naranjo Sofía , Kim Hye Kyong , Rodríguez-López María José , Chen Gang , Glauser Gaétan , Leiss Kirsten A. , Klinkhamer Peter G. L. 
Published: October 2019

AbstractUltraviolet (UV) radiation has emerged as an environmental cue with potential uses to enhance plant protection against arthropod pests in agriculture. UV can augment constitutive and inducible plant defenses against herbivorous arthropods. Here we investigated whether application of supplemental UV to chrysanthemum (Chrysanthemum × morifolium Ramat) cuttings during their rooting phase enhanced plant resistance to an important insect pest, Western flower thrips (Frankliniella occidentalis). For this, we analyzed how several daily UV exposure times affected plant damage by thrips on three different chrysanthemum cultivars. The most effective UV dose and responsive cultivar were further used to determine the UV effects on host plant preference by thrips, leaf metabolome and the induction of jasmonic acid (JA)-associated defenses. Our results showed that while short UV daily exposure times increased chrysanthemum resistance to thrips, longer exposure times had the opposite effect. Furthermore, we showed that UV-mediated induction of chrysanthemum resistance to thrips was genotype dependent and can persist after the end of the of the UV treatment. Yet, this induction was not transferred to the next generation from mother plants to cuttings. Nontargeted metabolomic, enzymatic and hormone analyses further revealed that UV slightly affected the leaf metabolome of chrysanthemum plants, and it enhanced the induction of JA-associated signaling after thrips infestation. Taken together, our results suggest that supplemental UV might modulate both constitutive and inducible chrysanthemum defenses against thrips.
“Offensiphobia” is a Red Herring: On the Problem of Censorship and Academic Freedom
Authors: Cross Ben , Richardson-Self Louise 
Published: October 2019

AbstractIn a recent article, J. Angelo Corlett criticises what he takes to be the ‘offensiphobic’ practices characteristic of many universities. The ‘offensiphobe’, according to Corlett, believes that offensive speech ought to be censured precisely because it offends. We argue that there are three serious problems with Corlett’s discussion. First, his criticism of ‘offensiphobia’ misrepresents the kinds of censorship practiced by universities; many universities may in some way censure speech which they regard as offensive, but this is seldom if ever a manifestation of ‘offensiphobia’. Second, we attempt to reconstruct Corlett’s criticism of ‘offensiphobia’ as a criticism of the practice of censuring hate speech, and show that this argument is unsuccessful. Third, we offer some brief reflections on how labelling universities as ‘offensiphobic’ is especially problematic in light of the current climate of political interference in university research and teaching.
Prioritising sites for pollinators in a fragmented coastal nectar habitat network in Western Europe
Authors: Paterson George B. , Smart Gill , McKenzie Paul , Cook Sally 
Published: October 2019

AbstractContextHabitat loss and fragmentation contribute significantly to pollinator decline and biodiversity loss globally. Conserving high quality habitats whilst restoring and connecting remnant habitat is critical to halt such declines.ObjectivesWe quantified the connectivity of pollinator habitats for a generic focal species (GFS) which represented three groups of pollinators in an existing coastal nectar habitat network. Subsequently, in partnership with a conservation agency, we modelled an improved landscape that identified priority habitat patches to increase connectivity for pollinators.MethodsWe selected 4260 pollinator habitats along an 80 km section of coastland in Scotland using Phase 1 habitat data. A GFS represented three vulnerable European pollinator groups while graph theory and spatial metrics were used to identify optimal sites that could enhance habitat connectivity.ResultsHigher dispersing species experienced greater habitat connectivity in the improved landscape and habitat availability increased substantially in response to small increases in habitat. The improved landscape revealed important habitat patches in the existing landscape that should be protected and developed.ConclusionsOur findings highlight that optimal landscapes can be designed through the integration of habitat data with spatial metrics for a GFS. By adopting this novel approach, conservation strategies can be targeted in an efficient manner to conserve at-risk species and their associated habitats. Integrating these design principles with policy and practice could enhance biodiversity across Europe.
Psychometric evaluation of the Participation and Activity Inventory for Children and Youth (PAI-CY) 0–2 years with visual impairment
Authors: Elsman Ellen B. M. , van Nispen Ruth M. A. , van Rens Gerardus H. M. B. 
Published: October 2019

Abstract

Purpose
To identify and monitor the developmental and participation needs of visually impaired (VI) children, the Participation and Activity Inventory for Children and Youth (PAI-CY) has recently been developed involving end-users as stakeholders. The aim was to investigate psychometric properties of the PAI-CY for children between 0 and 2 years.

Methods
Responses from 115 parents were included in item analyses, after which a combination of classical test theory and item response theory (IRT) was used. Internal consistency, known-group validity, and test–retest reliability at item and scale level were investigated.

Results
After deleting four items, the PAI-CY met IRT assumptions, i.e., unidimensionality, local independence, and monotonicity, and satisfactory model fit was obtained. Participants with more severe VI and comorbidity scored significantly worse than those with less severe VI and without comorbidity, supporting known-group validity. Satisfactory internal consistency and test–retest reliability were obtained (Cronbach’s alpha 0.95, kappa 0.60–0.91, ICC 0.920).

Conclusions
The PAI-CY 0–2 years has acceptable psychometric properties and can be used to systematically assess and monitor developmental and participation needs of very young children with VI from parents’ perspectives in low vision practice and research. Confirmation of psychometric properties is necessary, possibly facilitating further item reduction, increased precision, and improved user-friendliness.
Marginal Abatement Cost Curves for Water Scarcity Mitigation under Uncertainty
Authors: Sjöstrand Karin , Lindhe Andreas , Söderqvist Tore , Dahlqvist Peter , Rosén Lars 
Published: October 2019

AbstractAs water scarcity and drought become more common, planning to avoid their consequences becomes crucial. Measures to prevent the impact of new climate conditions are expected to be extensive, costly and associated with major uncertainties. It is therefore necessary that policymakers and practitioners in both the public and private sector can compare possible mitigation measures in order to make economically rational investment decisions. For this to be successful, decision-makers need relevant decision support. This paper presents a novel approach of constructing marginal abatement cost curves for comparing water scarcity mitigation measures while taking the underlying uncertainties into account. Uncertainties in input variables are represented by probability distributions and calculations are performed using Monte Carlo simulations. This approach is applied on the island of Gotland, one of the most water-stressed parts of Sweden, to provide the first marginal abatement cost curve in Europe for water scarcity mitigation in which municipal, agricultural, industrial and household measures are compared. The results show that the agricultural measure of on-farm storage has the greatest potential to increase water availability on the island. Among municipal measures, increased groundwater extraction and desalination offer the greatest potential, although desalination is almost 25 times more costly per cubic meter. The most cost-effective measure is linked to hot water savings in the hotel industry. The approach presented provides a quantitative visualization of the financial trade-offs and uncertainties implied by different mitigation measures. It provides critical economic insights for all parties concerned and is thus an important basis for decision-making.
Assessment of short-term changes in street dust pollution with heavy metals in Lublin (E Poland)—levels, sources and risks
Authors: Zgłobicki Wojciech , Telecka Małgorzata , Skupiński Sebastian 
Published: October 2019

Abstract
Street dust forms as a result of the interaction of the atmosphere, lithosphere (pedosphere) and anthroposphere and can be regarded as an index of the condition of the environment in urban areas. At the end of the twentieth century, there was a significant decrease in heavy metal emissions in Europe, but not so intensive in Poland. The question arises: Is the intensity of pollution still decreasing? The study objective was to assess changes in street dust pollution with heavy metals in Lublin (E Poland) in the years 2013 and 2018. The sample collection sites (68) were located within streets with a varying intensity of motor traffic. Cd, Cr, Cu, Ni, Pb and Zn concentrations were determined in two dust fractions, 63–200 μm and < 63 μm, by means of an X-ray fluorescence spectrometer. The levels of street dust pollution with heavy metals, expressed both in absolute concentrations and geochemical indices, were lower in 2018 than those in 2013. The clearest decrease of concentration levels occurred within the main roads, in the 63–200 μm fraction for Cu and Cd, and in both fractions for Pb. The mean concentrations of the investigated metals, normalised to the background values, are in the following order for both fractions in 2013 and 2018: Zn > Cd > Cu > Cr > Pb > Ni. Metals form the following order for Igeo and EF: Zn > Cd > Cu > Pb > Cr > Ni. This order is slightly different for the ecological risk factor: Cd > Cu > Pb > Zn > Cr > Ni. In general, street dust in Lublin does not show contamination with Cr, Ni and Pb. Igeo and EF indices show moderate levels of pollution with Cu, Cd and Zn.
Near-Field-Induced Femtosecond Breakdown of Plasmonic Nanoparticles
Authors: Nagy Benedek J. , Pápa Zsuzsanna , Péter László , Prietl Christine , Krenn Joachim R. , Dombi Péter 
Published: October 2019

Abstract
We studied the evolution of femtosecond breakdown in lithographically produced plasmonic nanoparticles with increasing laser intensity. Localized plasmons were generated with 40-fs laser pulses with up to 1.4 × 1012 W/cm2 peak intensity. The damage morphology shows substantial variation with intensity, starting with the detachment of hot spots and stochastic nanoparticle removal. For higher intensities, we observe precise nanolithographic mapping of near-field distributions via ablation. The common feature of these phenomena is the central role played by the single plasmonic hot spot of the triangular nanoparticles used. We also derive a damage threshold value from stochastic damage trends on the arrays fostering the optimization of novel nanoarchitectures for nonlinear plasmonics.
Exploring Correlations Between Properties Using Artificial Neural Networks
Authors: Zhang Yiming , Evans Julian R. G. , Yang Shoufeng 
Published: October 2019

Abstract
The traditional aim of materials science is to establish the causal relationships between composition, processing, structure, and properties with the intention that, eventually, these relationships will make it possible to design materials to meet specifications. This paper explores another approach. If properties are related to structure at different scales, there may be relationships between properties that can be discerned and used to make predictions so that knowledge of some properties in a compositional field can be used to predict others. We use the physical properties of the elements as a dataset because it is expected to be both extensive and reliable and we explore this method by showing how it can be applied to predict the polarizability of the elements from other properties.
Three-Dimensional Additively Manufactured Microstructures and Their Mechanical Properties
Authors: Rodgers Theron M. , Lim Hojun , Brown Judith A. 
Published: October 2019

Abstract
Metal additive manufacturing (AM) allows for the freeform creation of complex parts. However, AM microstructures are highly sensitive to the process parameters used. Resulting microstructures vary significantly from typical metal alloys in grain morphology distributions, defect populations and crystallographic texture. AM microstructures are often anisotropic and possess three-dimensional features. These microstructural features determine the mechanical properties of AM parts. Here, we reproduce three “canonical” AM microstructures from the literature and investigate their mechanical responses. Stochastic volume elements are generated with a kinetic Monte Carlo process simulation. A crystal plasticity-finite element model is then used to simulate plastic deformation of the AM microstructures and a reference equiaxed microstructure. Results demonstrate that AM microstructures possess significant variability in strength and plastic anisotropy compared with conventional equiaxed microstructures.
Anti-conformism in the Threshold Model of Collective Behavior
Authors: Grabisch Michel , Li Fen 
Published: October 2019

AbstractWe provide a detailed study of the threshold model, where both conformist and anti-conformist agents coexist. Our study bears essentially on the convergence of the opinion dynamics in the society of agents, i.e., finding absorbing classes, cycles, etc. Also, we are interested in the existence of cascade effects, as this may constitute an undesirable phenomenon in collective behavior. We divide our study into two parts. In the first one, we basically study the threshold model supposing a fixed complete network, where every one is connected to every one, like in the seminal work of Granovetter. We study the case of a uniform distribution of the threshold, of a Gaussian distribution, and finally give a result for arbitrary distributions, supposing there is one type of anti-conformist. In a second part, we suppose that the neighborhood of an agent is random, drawn at each time step from a distribution. We distinguish the case where the degree (number of links) of an agent is fixed, and where there is an arbitrary degree distribution. We show the existence of cascades and that for most societies, the opinion converges to a chaotic situation.
A simple and accurate PCR method for detection of genetically modified rice
Authors: Safaei Payam , Aghaee Ebrahim Molaee , Khaniki Gholamreza Jahed , Afshari Setareh Agha Kuchak , Rezaie Sassan 
Published: October 2019

Abstract

Background
Legislation regulating for labeling and use of genetically modified (GM) crops are increased considerably worldwide in order to health and safety assurance of consumers. For this purpose, a polymerase chain reaction (PCR) method has been developed for detection of GM rice in people’s food diet.

Methods
In this study, eighty-one non-labeled rice samples were collected randomly from different market sites of Tehran, Iran. In order to analysis, rice genomic DNA was extracted using MBST DNA extraction kit and subsequently, sucrose phosphate synthase (SPS) gene was used to confirm the quality of extracted DNA. Then, cauliflower mosaic virus (CaMV) 35S promoter and Agrobacterium nopaline synthase (NOS) terminator were selected as screening targets for detection of GM rice sequences by PCR.

Results
According to our results, 2 out of 81 (2.4%) samples tested were positive for CaMV 35S promoter while no positive result was detected for NOS terminator.

Conclusion
The obtained data indicated that this method is capable to identify the GM rice varieties. Furthermore, it can demonstrate the possibility of the presence of GM rice in Tehran’s market, thus putting emphasis on the requirement for developing a precise approach to evaluate this product.
Working-class entrepreneurialism: Perceptions, aspirations, and experiences of petty entrepreneurship among male manual workers in Turkey
Authors: Birelma Alpkan 
Published: October 2019

AbstractThis article examines working-class entrepreneurialism in Turkey from a comparative perspective. Based on ethnographic fieldwork conducted in a working-class neighborhood of İstanbul, the article focuses on the perceptions, aspirations, and entrepreneurial attempts of manual workers employed in formal jobs. It aims to contribute to the understudied literature on working-class entrepreneurialism, which is often overlooked or underestimated by the critical research on labor and the working class. First, the article demonstrates that the level of entrepreneurialism among manual workers is rather high. Alongside revealing the popularity of aspirations for self-employment and the working-class roots of many self-employed individuals, I present an ethnographic account of five workers’ transition from wage work to self-employment. Second, the article finds that a colloquial phrase, “el işi” or “a stranger’s business,” is widely used to refer to wage work. I argue that this phrase perfectly manifests the popular resentment felt toward wage labor in a social milieu where self-employment seems accessible. Finally, by drawing on a review of a scattered set of studies, I claim that entrepreneurialism among working-class men seems to be quite common, especially in peripheral countries.
Does Political Corruption Impede Firm Innovation? Evidence from the United States
Authors: Huang Qianqian , Yuan Tao 
Published: October 2019

AbstractWe examine how local political corruption affects firm innovation in the United States. We find that firms located in highly corrupt areas are less innovative as measured by their patenting activities. The results are robust to the inclusion of a broad set of regional characteristics, instrumental variable analysis, matching analysis, difference-in-differences test, and alternative proxies for local corruption. Further analysis shows that reduced innovation incentives due to high extortion risk and decreased threat of competition could be the possible economic channels through which corruption affects innovation. Overall, our results indicate that local political corruption impedes corporate innovation in the United States.
A New Gantry-Tau-Based Mechanism Using Spherical Wrist and Model Predictive Control-Based Motion Cueing Algorithm
Authors: Qazani Mohammad Reza Chalak , Asadi Houshyar , Nahavandi Saeid 
Published: October 2019

SUMMARYThe 3 degree-of-freedom Gantry-Tau manipulator with the addition of the spherical wrist mechanism which is called Gantry-Tau-3R is designed as a high-G simulation-based motion platform (SBMP) with the capability of generating the large linear and angular displacement. The combination of both parallel and serial manipulator in newly designed Gantry-Tau-3R mechanism improves the ability of the mechanism to regenerate larger motion signals with higher linear acceleration and angular velocity. The high-frequency signals are reproduced using the parallel part of the mechanism, and sustainable low-frequency accelerations are regenerated via the serial part due to the larger rotational motion capability, which will be used through motion cueing algorithm tilt coordination channel. The proportional integral derivative (PID) and fuzzy incremental controller (FIC) are developed for the proposed mechanism to show the high path tracking performance as a motion platform. FIC reduces the motion tracking error of the newly designed Gantry-Tau-3R and increases the motion fidelity for the users of the proposed SBMP. The proposed method is implemented using Matlab/Simulink software. Finally, the results demonstrate the accurate motion signal generation using linear model predictive motion cues with a fuzzy controller, which is not possible using the common parallel and serial manipulators.
Caring Values and the Value of Care: Women, Maternalism and Caring Work in the Czech Republic
Authors: Read Rosie 
Published: October 2019

AbstractThis article examines maternalism in the Czech Republic by exploring how waged and unwaged forms of caring work were framed through discourses of women's innately caring nature in the late twentieth century. Present-day hospital volunteering programmes, which bring female, lay volunteers onto hospital wards to provide unwaged care to patients, are inscribed by maternalist tropes historically associated with domestic work and family care, rather than the neutral expertise associated with female waged care workers in public, institutional settings. The article assesses the contemporary reinvention of maternalist discourses and their capacity to mobilise unwaged caring labour.