bluuebunny/crossref_metadata_2025 huggingface dataset:
De smokkel van Syrische migranten naar Nederland
Authors: van der Velden Sacha
Published: September 2017

The smuggling of Syrian migrants to the Netherlands

In this article the following question is answered. What is the modus operandi of human smugglers that smuggle Syrian migrants to the Netherlands in de period 2014-2016? Content analysis of police and Royal Marechaussee files, interviews with experts and Syrian migrants and literature research has revealed that: Syrian migrants in first instance use the South-Eastern route overseas from Turkey to Greece, for which they pay 1200-4000 euro to a smuggler, depending on the quality of the boat. From Greece some travel over land on the Balkan route. Several means of transportation are used on this route, with smugglers asking prices ranging from tens to hundreds of euros. Also they travel large parts on their own. Others are smuggled by airplane, for which they need (false) documentation. Smuggling by plane tends to be successful when departing from a small airfield, when made a detour through visa-free countries and/or a good quality (false) travel document. Prices range from 2.000 to 14.000 euros. Migrants mostly pay cash through irregular ‘banks’, a third party that plays an important facilitating role within several smuggling networks. These networks consist of several cells, with in these cells leaders and ‘employees’ and are located on migration routes and ‘hubs’ like Istanbul, Athens and Milan. The South-Eastern route is mostly in hands of Turkish criminal networks, and the continuation of the journey by airplane or through Milan over land is in hands of Syrian Dutch smugglers, the Balkan route seems to be mainly operated by individual smugglers. Within these smuggling networks the most important roles are: the recruiter, who is responsible for making contact with migrants on migration hubs or social media. The organizer/leader who coordinates activities along the smuggling route and calls employees to account. And finally the driver. Responsible for transporting the migrants and instructing them. Based on these findings several recommendations were made that can be found in this article or full research rapport.
Eer op de vlucht
Authors: Janssen Janine , Sanberg Ruth
Published: September 2017

Honour on the run On honour based violence in Dutch asylum centers

In this exploratory study, two questions are addressed. Firstly, examining police files on honour based violence (HBV), do we find conflicts that take place in asylum centers, and if so, what can we learn from those files? Secondly, to what extent can these police files provide insight into the role of HBV in the lives of refugees in the Netherlands? What are the possibilities and limitations and how can they be dealt with in future research?

We apply the importation and deprivation models within total institutions on asylum centers and zoom in on honour based conflicts within these centers. The conflicts revolved around family life and morality issues, and resulted in both threats and physical violence. Among younger victims, their choice of partner was the main stumbling block, while later in the course of life conflicts arose from divorces and new relationships. Distinguishing between importation and deprivation factors within these HBV cases turned out to be difficult for two reasons. Firstly, within Dutch policy HBV is primarily viewed as violence within (extended) families and often originating from conflicts regarding partner choice and morality. This focus corresponds to importation rather than deprivation. Secondly, many cases showed interaction between the two factors, for example the many different backgrounds of the inhabitants of the asylum center can be viewed as both importation and deprivation. Future research should look into (a combination of) different data sources to shed light on the role of deprivation, because deprivational factors are more susceptible for (short term) policy measures to help prevent unnecessary stress and causes of conflict, whether honour based or not.
The use of selected neutrophil protein plasma concentrations in the diagnosis of Crohn’s disease and ulcerative colitis – a preliminary report
Authors: Pawlica-Gosiewska Dorota , Solnica Bogdan , Gawlik Katarzyna , Cibor Dorota , Mach Tomasz , Fedak Danuta , Owczarek Danuta
Published: September 2017

Background: Difficulties in diagnosis of inflammatory bowel disease (IBD) motivate the search for new diagnostic tools, including laboratory tests. The aim of this study was to evaluate concentrations of the neutrophil (NEU) proteins leukocyte elastase (HLE-α1AT), lactoferrin and calprotectin as potential biomarkers used in the diagnosis and assessment of clinical activity of Crohn’s disease (CD) and ulcerative colitis (UC).Material/Methods: The study included 27 patients with CD, 33 patients with UC and 20 healthy controls. Plasma concentrations of calprotectin, lactoferrin and HLE-α1AT were measured using ELISA.Results: In patients with CD higher concentrations of HLE-α1AT (64.3±43.1 vs. 30.1±7.7 ng/l, P&lt;0.001), calprotectin (151.6±97.8 vs. 69.9±22.1 ng/l, P&lt;0.001) and lactoferrin (243.2±102.0 vs. 129.7±32.7 ng/l, P&lt;0.001) than in the control group were found. In patients with UC higher plasma concentrations of HLE-α1AT (62.0±30.9 vs. 30.1±7.7 ng/l, P&lt;0.001), calprotectin (149.6±72.3 vs. 69.9±22.1 ng/l, P&lt;0.001) and lactoferrin (242.6±107.5 vs 129.7±32.7 ng/l, P&lt;0.001) than in the control group were found. HLE-α1AT/NEU and lactoferrin/NEU ratios in patients with UC were significantly higher compared with patients with CD. Calprotectin (P=0.010) and lactoferrin (P=0.023) levels were higher in patients with the active compared with inactive phase of CD.Conclusions: The diagnostic characteristics of plasma granulocyte protein concentrations indicate the usefulness of these tests in the diagnosis of IBD. Higher HLE-α1AT and lactoferrin/NEU ratios in patients with UC than with CD may suggest the usefulness of these ratios in differential diagnostics. Plasma calprotectin and lactoferrin levels may be useful in CD activity assessment.
The Knowledge Transfer from Universities to Tourism Companies – Identification of Barriers and Opportunities for their Reduction
Authors: Olszewski Marcin , Walas Bartłomiej
Published: September 2017

Purpose. The purpose of this article is to identify the role of universities as sources of knowledge, and to recognise barriers in knowledge transfer from universities to tourism companies. Moreover, the activities, which can stimulate such cooperation were also proposed. Method. In the article, a critical review of the literature is applied. Findings. The key barriers to the knowledge transfer are identified. The barriers are grouped into the following categories: companies related barriers, universities related barriers and relational barriers. Moreover, author’s own proposals of the activities contributing to intensification of the knowledge transfer from academia are presented. Research and conclusion limitations. Both the identified barriers, as well as proposals to reduce them have not been empirically verified in the Polish context. Practical implications. In the article, the recommendations of activities stimulating knowledge transfer for universities, tourism enterprises and the public administration sector are presented. Originality. The analysis of knowledge transfer determinants, takes into account both the perspective of universities, businesses, as well as the sphere of relations between them (the concept of the proximity in many dimensions). This comprehensive approach is not found in previous publications. The multithreading of the part containing practical implications. Type of paper. Review article.
A Pageant Politicized: Women’s Liberation, Miss Black America and Intersectionality in 1968
Authors: Cormier Haley Sierra
Published: September 2017

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Age, growth, mortality and reproductive seasonality of jolthead porgy, Calamus bajonado, from Florida waters
Authors: Burton Michael L. , Potts Jennifer C. , Page Jordan , Poholek Ariel
Published: September 2017

Ages of jolthead porgy (Calamus bajonado Schneider 1801) (n = 635) from Florida commercial and recreational fisheries from 2008–2016 were determined using sectioned sagittal otoliths. We determined, using edge-type analysis, that opaque zones were annular, forming March–June (peaking in April). Jolthead porgy ranged from 1–13 years, and the largest fish measured 680 mm TL (total length, mm). Body size relationships for jolthead porgy were TL = 1.09FL + 20.44 (n = 622, r2 = 0.99), FL = 0.90 TL –14.26 (n = 622, r2 = 0.99), and W = 1.1 × 10−5 TL3.06 (n = 577), where W is total weight (grams, g) and FL is fork length (mm). The von Bertalanffy growth equation for jolthead porgy was Lt = 737(1 − e−0.14(t+2.02)) (n = 635). Point estimate of natural mortality was M = 0.32, while age-specific estimates of M ranged from 0.58–0.17 y−1 for ages 1–13. Catch curve analysis estimated the instantaneous rate of total mortality Z = 0.70, while instantaneous rate of fishing mortality F was 0.38. Macroscopic staging of female gonads indicated the presence of hydrated oocytes from December–March, and GSI data indicates that peak spawning in females occurs during March. This study presents the first published findings of life history parameters for jolthead porgy from the Atlantic waters off the southeastern United States.
Exploiting off‐targeting in guide‐RNAs for CRISPR systems for simultaneous editing of multiple genes
Authors: Ferreira Raphael , Gatto Francesco , Nielsen Jens
Published: September 2017

Bioinformatics tools to design guide‐RNAs (gRNAs) in Clustered Regularly Interspaced Short Palindromic Repeats systems mostly focused on minimizing off‐targeting to enhance efficacy of genome editing. However, there are circumstances in which off‐targeting might be desirable to target multiple genes simultaneously with a single gRNA. We termed these gRNAs as promiscuous gRNAs. Here, we present a computational workflow to identify promiscuous gRNAs that putatively bind to the region of interest for a defined list of genes in a genome. We experimentally validated two promiscuous gRNA for gene deletion, one targeting FAA1 and FAA4 and one targeting PLB1 and PLB2, thus demonstrating that multiplexed genome editing through design of promiscuous gRNA can be performed in a time and cost‐effective manner.
MESSENGER Observations of Magnetotail Loading and Unloading: Implications for Substorms at Mercury
Authors: Imber S. M. , Slavin J. A.
Published: September 2017

We present the first statistical study of loading and unloading of magnetic flux in Mercury's magnetotail. These events describe the global circulation of magnetic flux through the magnetosphere and provide strong evidence that terrestrial‐type substorms take place at Mercury. 438 events were identified over the 4 years of the MErcury Surface, Space ENvironment, GEochemistry, and Ranging (MESSENGER) mission by a gradual, short‐lived increase in the magnetotail lobe magnetic field strength, coincident with an outward flaring of the magnetotail. Substorm duration ranged from tens of seconds to several minutes, with a median of 195 s and a mean of 212 s. The median amplitude of lobe magnetic field increase was ~11.5 nT, which represents an increase of 23.4% on the background lobe field strength, compared with ~10% for terrestrial substorms. The magnetotail lobes were found to contain ~2–3 MWb of magnetic flux based on 1031 tail passes, with a mean of 2.52 MWb and a standard deviation of 0.48 MWb. An estimate of the change in open flux content during the loading phase of each substorm ranged from 0.08 to 3.7 MWb with a mean value of 0.69 MWb and a standard deviation of 0.38 MWb. These changes in open flux content are an underestimate as the change in magnetotail radius during the events was not accounted for. The maximum lobe flux content during each substorm (~3 MWb) represented ~40% of the total available magnetic flux in the system (~7.5 MWb). During terrestrial substorms, the maximum lobe magnetic flux content is ~10–12% of the total flux from the dipole. A typical substorm at Mercury therefore cycles through a significantly larger fraction of the available magnetic flux than all but the largest substorms at the Earth.
Rheological Weakening of Olivine + Orthopyroxene Aggregates Due To Phase Mixing: Part 2. Microstructural Development
Authors: Tasaka Miki , Zimmerman Mark E. , Kohlstedt David L. , Stünitz Holger , Heilbronner Renée
Published: September 2017

To understand the processes involved in phase mixing during deformation and the resulting changes in rheological behavior, we conducted torsion experiments on samples of iron‐rich olivine plus orthopyroxene. The experiments were conducted at a temperature, T, of 1200°C and a confining pressure, P, of 300 MPa using a gas‐medium deformation apparatus. Samples composed of olivine plus 26% orthopyroxene were deformed to outer radius shear strains up to γ ≈ 26. In samples deformed to lower strains of γ ≲ 4, elongated olivine and pyroxene grains form a compositional layering. Already by this strain, mixtures of small equant grains of olivine and pyroxene begin to develop and continue to evolve with increasing strain. The ratios of olivine to pyroxene grain size in deformed samples follow the Zener relationship, indicating that pyroxene grains effectively pin the grain boundaries of olivine and inhibit grain growth. Due to the reduction in grain size, the dominant deformation mechanism changes as a function of strain. The microstructural development forming more thoroughly mixed, fine‐grained olivine‐pyroxene aggregates can be explained by the difference in diffusivity among Me (Fe or Mg), O, and Si, with transport of MeO significantly faster than that of SiO2. These mechanical and associated microstructural properties provide important constraints for understanding rheological weakening and strain localization in upper mantle rocks.
Climatology and decadal variations in multicore structure of the North Pacific subtropical mode water
Authors: Liu Cong , Xie Shang‐Ping , Li Peiliang , Xu Lixiao , Gao Wendian
Published: September 2017

The pycnostad of the North Pacific subtropical mode water (STMW) often displays multiple vertical minima in the potential vorticity profile. Argo profile data from 2004 to 2015 are used to investigate interannual to decadal variations of the multicore structure. Climatologically, about 24% pycostads of STMW have multicore structure, and most of them distribute in the region west of 150°E. STMW cores are classified into three submodes—the cold, middle, and warm ones with potential temperatures of 16.0–17°C, 17–18°C, and 18–19.5°C, respectively. The Kuroshio Extension (KE) varies between stable and unstable states. The unstable KE with large meanders increases the subsurface stratification and shoals the winter mixed layer east of 150°E with warmer temperatures. There, the dominant STMW type varies from the cold single type in stable KE years (making up 72% of the profiles with STMW) to the middle single one (53%) in unstable years. The variation of the dominant STMW type in the region east of 150°E subsequently affects the multicore structure of STMW west of 150°E. In a broad region between 130°E and 180°E, profiles with STMW are fewer in unstable years but the proportion of multicore profiles increases among STMW profiles. This might be due to the split recirculation gyre with a chaotic KE.
Satellite observed salinity distributions at high latitudes in the Northern Hemisphere: A comparison of four products
Authors: Garcia‐Eidell Cynthia , Comiso Josefino C. , Dinnat Emmanuel , Brucker Ludovic
Published: September 2017

Global surface ocean salinity measurements have been available since the launch of SMOS in 2009 and coverage was further enhanced with the launch of Aquarius in 2011. In the polar regions where spatial and temporal changes in sea surface salinity (SSS) are deemed important, the data have not been as robustly validated because of the paucity of in situ measurements. This study presents a comparison of four SSS products in the ice‐free Arctic region, three using Aquarius data and one using SMOS data. The accuracy of each product is assessed through comparative analysis with ship and other in situ measurements. Results indicate RMS errors ranging between 0.33 and 0.89 psu. Overall, the four products show generally good consistency in spatial distribution with the Atlantic side being more saline than the Pacific side. A good agreement between the ship and satellite measurements was also observed in the low salinity regions in the Arctic Ocean, where SSS in situ measurements are usually sparse, at the end of summer melt seasons. Some discrepancies including biases of about 1 psu between the products in spatial and temporal distribution are observed. These are due in part to differences in retrieval techniques, geophysical filtering, and sea ice and land masks. The monthly SSS retrievals in the Arctic from 2011 to 2015 showed variations (within ∼1 psu) consistent with effects of sea ice seasonal cycles. This study indicates that spaceborne observations capture the seasonality and interannual variability of SSS in the Arctic with reasonably good accuracy.
High‐Altitude Aeolian Research on the Tibetan Plateau
Authors: Dong Zhibao , Hu Guangyin , Qian Guangqiang , Lu Junfeng , Zhang Zhengcai , Luo Wanyin , Lyu Ping
Published: September 2017

Aeolian processes and their role in desertification have been studied extensively at low elevations but have been rarely studied at high elevations in areas such as the Tibetan Plateau, where aeolian processes were active in the geologic past and remain active today. In this review, we summarize research that improves our understanding of aeolian processes on the Tibetan Plateau, including the distribution, characteristics, and provenance of aeolian sediments; the history of aeolian activity; aeolian geomorphology; and wind‐driven land degradation. Contemporary aeolian processes primarily occur in dry basins, in wide river valleys, on lakeshores, on mountain slopes, and on gravel pavements. Sediment characteristics suggest a local origin and provide interesting contrasts with those of China's Loess Plateau. The history of aeolian activity and its paleoclimatic implications, reconstructed based on aeolian archives, is short (mostly since the Late Glacial) and shows wide regional differences. Aeolian geomorphology is simple and suggests short formation time. Wind‐driven land degradation is less severe than previously thought, driven by different factors in different areas, and exhibited complex interactions with freeze‐thaw processes. Aeolian research has been conducted within the general framework of aeolian science but addresses issues specific to the Tibetan Plateau that arise due to the low air temperature, low air density, and the presence of a cryosphere. We propose six priorities for future research: aeolian physics, the effect of freeze‐thaw cycles, comparisons with other areas, regional differences, effects of wind‐driven land degradation, and integrated observation and monitoring.
Improved diagnosis of Parkinson's disease from a detailed olfactory phenotype
Authors: Gerkin Richard C. , Adler Charles H. , Hentz Joseph G. , Shill Holly A. , Driver‐Dunckley Erika , Mehta Shyamal H. , Sabbagh Marwan N. , Caviness John N. , Dugger Brittany N. , Serrano Geidy , Belden Christine , Smith Brian H. , Sue Lucia , Davis Kathryn J. , Zamrini Edward , Beach Thomas G.
Published: September 2017

Objective

To assess the predictive potential of the complete response pattern from the University of Pennsylvania Smell Identification Test for the diagnosis of Parkinson's disease.

Methods

We analyzed a large dataset from the Arizona Study of Aging and Neurodegenerative Disorders, a longitudinal clinicopathological study of health and disease in elderly volunteers. Using the complete pattern of responses to all 40 items in each subject's test, we built predictive models of neurodegenerative disease, and we validated these models out of sample by comparing model predictions against postmortem pathological diagnosis.

Results

Consistent with anatomical considerations, we found that the specific test response pattern had additional predictive power compared with a conventional measure – total test score – in Parkinson's disease, but not Alzheimer's disease. We also identified specific test questions that carry the greatest predictive power for disease diagnosis.

Interpretation

Olfactory ability has typically been assessed with either self‐report or total score on a multiple choice test. We showed that a more accurate clinical diagnosis can be made using the pattern of responses to all the test questions, and validated this against the “gold standard” of pathological diagnosis. Information in the response pattern also suggests specific modifications to the standard test that may optimize predictive power under the typical clinical constraint of limited time. We recommend that future studies retain the individual item responses for each subject, and not just the total score, both to enable more accurate diagnosis and to enable additional future insights.
Direct Laser Writing of Superhydrophobic PDMS Elastomers for Controllable Manipulation via Marangoni Effect
Authors: Wang Wei , Liu Yu‐Qing , Liu Yan , Han Bing , Wang Huan , Han Dong‐Dong , Wang Jian‐Nan , Zhang Yong‐Lai , Sun Hong‐Bo
Published: September 2017

Direct light‐to‐work conversion enables manipulating remote devices in a contactless, controllable, and continuous manner. Although some pioneering works have already proven the feasibility of controlling devices through light‐irradiation‐induced surface tension gradients, challenges remain, including the flexible integration of efficient photothermal materials, multifunctional structure design, and fluidic drag reduction. This paper reports a facile one‐step method for preparing light‐driven floating devices with functional surfaces for both light absorption and drag reduction. The direct laser writing technique is employed for both arbitrary patterning and surface modification. By integrating the functional layer at the desired position or by designing asymmetric structures, three typical light‐driven floating devices with fast linear or rotational motions are demonstrated. Furthermore, these devices can be driven by a variety of light sources including sunlight, a filament lamp, or laser beams. The approach provides a simple, green, and cost‐effective strategy for building functional floating devices and smart light‐driven actuators.
Carbon Nanotube Based Inverted Flexible Perovskite Solar Cells with All‐Inorganic Charge Contacts
Authors: Luo Qiang , Ma He , Hao Feng , Hou Qinzhi , Ren Jing , Wu Lili , Yao Zhibo , Zhou Yu , Wang Ning , Jiang Kaili , Lin Hong , Guo Zhanhu
Published: September 2017

Organolead halide perovskite solar cells (PSC) are arising as promising candidates for next‐generation renewable energy conversion devices. Currently, inverted PSCs typically employ expensive organic semiconductor as electron transport material and thermally deposited metal as cathode (such as Ag, Au, or Al), which are incompatible with their large‐scale production. Moreover, the use of metal cathode also limits the long‐term device stability under normal operation conditions. Herein, a novel inverted PSC employs a SnO2‐coated carbon nanotube (SnO2@CSCNT) film as cathode in both rigid and flexible substrates (substrate/NiO‐perovskite/Al2O3‐perovskite/SnO2@CSCNT‐perovskite). Inverted PSCs with SnO2@CSCNT cathode exhibit considerable enhancement in photovoltaic performance in comparison with the devices without SnO2 coating owing to the significantly reduced charge recombination. As a result, a power conversion efficiency of 14.3% can be obtained on rigid substrates while the flexible ones achieve 10.5% efficiency. More importantly, SnO2@CSCNT‐based inverted PSCs exhibit significantly improved stability compared to the standard inverted devices made with silver cathode, retaining over 88% of their original efficiencies after 550 h of full light soaking or thermal stress. The results indicate that SnO2@CSCNT is a promising cathode material for long‐term device operation and pave the way toward realistic commercialization of flexible PSCs.
Transparent and Flexible Nacre‐Like Hybrid Films of Aminoclays and Carboxylated Cellulose Nanofibrils
Authors: Liu Yingxin , Yu Shu‐Hong , Bergström Lennart
Published: September 2017

Nacre and other biological composites are important inspirations for the design and fabrication of multifunctional composite materials. Transparent, strong, and flexible hybrid films of aminoclays (AC) and carboxylated cellulose nanofibrils (CNF) with a nacre‐like microstructure at AC contents up to 60 wt% are prepared. The high transmittance of visible light is attributed to the high homogeneity of the hybrid films and to the relatively small refractive index contrast between the CNF‐based matrix and synthetic AC. The strength and strain to failure of the hybrids are significantly higher than biogenic nacre and other nacre‐mimicking nanocellulose‐based materials, e.g., montmorillonite‐CNF and graphene oxide‐CNF composite films. The excellent mechanical properties are related to the ionic bonds between the negatively charged carboxylic groups on the CNF and the positively charged amine groups on the AC nanoparticles. This work illustrates the significance of tailoring the interactions between small clay particles and biopolymers in multifunctional materials with potential applications as printable barrier coatings and substrates for optoelectronics.
Dual Functionalization of Liquid‐Exfoliated Semiconducting 2H‐MoS2 with Lanthanide Complexes Bearing Magnetic and Luminescence Properties
Authors: McAdams Simon G. , Lewis Edward A. , Brent Jack R. , Haigh Sarah J. , Thomas Andrew G. , O'Brien Paul , Tuna Floriana , Lewis David J.
Published: September 2017

Liquid exfoliated, atomically thin semiconducting transition metal dichalcogenides (TMDs), as inorganic equivalents of graphene, have attracted great interest due to their distinctive physical, optoelectronic, and chemical properties. Functionalization of 2D TMDs brings new prospects for applications in optoelectronics, quantum technologies, catalysis, and medicine. In this report, dual functionalization of 2D semiconducting 2H‐MoS2 nanosheets through simultaneous incorporation of magnetic and luminescent properties is demonstrated. A facile method is proposed for tuning the properties of the TDM semiconductors and accessing multimodal platforms, consisting in covalent grafting of lanthanide complexes onto the surface of 2D TMDs. Dual functionalization of liquid‐exfoliated MoS2 nanosheets is demonstrated simultaneously with both europium (III) and gadolinium (III) complexes to form a colloidally stable luminescent (with millisecond lifetimes) and paramagnetic MoS2‐based nanohybrid material. This work is the first example of transition metal dichalcogenide nanosheets functionalized with preformed lanthanide complexes. These findings open new prospects for covalent functionalization of TMDs with molecular species bearing specific functionalities as a means to tune the optoelectronic properties of the semiconductors, in order to create advanced materials and devices with a wide range of functionalities.
Thermosensitive Hydrogel Containing Doxycycline Exerts Inhibitory Effects on Abdominal Aortic Aneurysm Induced By Pancreatic Elastase in Mice
Authors: Yu Maomao , Dong Anjie , Chen Cong , Xu Shuxin , Cao Yini , Liu Shu , Zhang Qiang , Qi Rong
Published: September 2017

Doxycycline (DOX) is reported to exert therapeutic effects against abdominal aortic aneurysm (AAA), a severe degenerative disease. In this study, a DOX hydrogel formulation of DOX/PECTgel is studied, and its phase transition behavior and in vitro release profiles are explored. In addition, the anti‐AAA effects and bioavailability of DOX/PECTgel are evaluated in an elastase induced AAA mouse model. The results show that the phase transition temperature of 30% poly(e‐caprolactone‐co‐1,4,8‐trioxa[4.6]spiro‐9‐undecanone) (PECT) solution is above 34 °C. In vitro release profiles of DOX/PECTgel indicate a fast release of DOX at the first two days, followed by a slow and sustained release for 14 d. In vivo single‐dose single subcutaneous injection of DOX/PECTgel containing 8.4 or 4.2 mg mL−1 DOX presents comparatively preventive effects on AAA, compared to intraperitoneal injections of DOX alone at a dose of 15 mg kg−1 for seven injections, while DOX bioavailability of the DOX/PECTgel treated groups is 1.39 times or 1.19 times of the DOX alone treated group, respectively.
Liquid Quinones for Solvent‐Free Redox Flow Batteries
Authors: Shimizu Akihiro , Takenaka Keisuke , Handa Naoyuki , Nokami Toshiki , Itoh Toshiyuki , Yoshida Jun‐Ichi
Published: September 2017

Liquid benzoquinone and naphthoquinone having diethylene glycol monomethyl ether groups are designed and synthesized as redox active materials that dissolve supporting electrolytes. The Li‐ion batteries based on the liquid quinones using LiBF4/PC show good performance in terms of voltage, capacity, energy efficiency, and cyclability in both static and flow modes. A battery is constructed without using intentionally added organic solvent, and its high energy density (264 W h L−1) demonstrates the potential of solvent‐free organic redox flow batteries using liquid active materials.
Wearable Large‐Scale Perovskite Solar‐Power Source via Nanocellular Scaffold
Authors: Hu Xiaotian , Huang Zengqi , Zhou Xue , Li Pengwei , Wang Yang , Huang Zhandong , Su Meng , Ren Wanjie , Li Fengyu , Li Mingzhu , Chen Yiwang , Song Yanlin
Published: September 2017

Dramatic advances in perovskite solar cells (PSCs) and the blossoming of wearable electronics have triggered tremendous demands for flexible solar‐power sources. However, the fracturing of functional crystalline films and transmittance wastage from flexible substrates are critical challenges to approaching the high‐performance PSCs with flexural endurance. In this work, a nanocellular scaffold is introduced to architect a mechanics buffer layer and optics resonant cavity. The nanocellular scaffold releases mechanical stresses during flexural experiences and significantly improves the crystalline quality of the perovskite films. The nanocellular optics resonant cavity optimizes light harvesting and charge transportation of devices. More importantly, these flexible PSCs, which demonstrate excellent performance and mechanical stability, are practically fabricated in modules as a wearable solar‐power source. A power conversion efficiency of 12.32% for a flexible large‐scale device (polyethylene terephthalate substrate, indium tin oxide‐free, 1.01 cm2) is achieved. This ingenious flexible structure will enable a new approach for development of wearable electronics.
Controlling Magnetic Properties at BiFe1−xMnxO3/La2/3Ca1/3MnO3 Interfaces by Tuning the Spatial Distribution of Interfacial Electronic States
Authors: Chen Mingjing , Ning Xingkun , Hu Xiaobing , Sun Jirong , Fu Nian , Li Yanxi , Wang Jianglong , Wang Shufang , Fu Guangsheng
Published: September 2017

One major challenge in engineering the magnetism of oxide heterostructures is controlling orbital reconstruction by tuning the charge transfer effect. This paper investigates the forward and backward charge transfer effect and the induced magnetic properties of BiFe1−xMnxO3/La2/3Ca1/3MnO3 heterostructures. Interfacial ferromagnetism is found to be qualitatively tunable by tuning the spatial distribution of interfacial electronic states. The subtle balance of magnetic coupling between FeOMn and MnOMn changed as the electronic structures are modified by charge transfer. The ions' valence is strongly correlated with the interfacial magnetic properties, extending the concept of the charge degrees of freedom affecting the magnetic coupling properties.
Survival beyond the perinatal period expands the phenotypes caused by mutations in GLE1
Authors: Said Edith , Chong Jessica X. , Hempel Maja , Denecke Jonas , Soler Paul , Strom Tim , Nickerson Deborah A. , Kubisch Christian ,   University of Washington Center for Mendelian Genomics, Bamshad Michael J. , Lessel Davor
Published: September 2017

Mutations in GLE1 underlie Lethal Congenital Contracture syndrome (LCCS) and Lethal Arthrogryposis with Anterior Horn Cell Disease (LAAHD). Both LCCS and LAAHD are characterized by reduced fetal movements, congenital contractures, and a severe form of motor neuron disease that results in fetal death or death in the perinatal period, respectively. We identified bi‐allelic mutations in GLE1 in two unrelated individuals with motor delays, feeding difficulties, and respiratory insufficiency who survived beyond the perinatal period. Each affected child had missense variants predicted to result in amino acid substitutions near the C‐terminus of GLE1 that are predicted to disrupt protein–protein interaction or GLE1 protein targeting. We hypothesize that mutations that preserve function of the coiled‐coil domain of GLE1 cause LAAHD whereas mutations that abolish the function of the coiled‐coil domain cause LCCS. The phenotype of LAAHD is now expanded to include multiple individuals surviving into childhood suggesting that LAAHD is a misnomer and should be re‐named Arthrogryposis with Anterior Horn Cell Disease (AAHD).
A novel missense variant in the GLI3 zinc finger domain in a family with digital anomalies
Authors: Crapster J. Aaron , Hudgins Louanne , Chen James K. , Gomez‐Ospina Natalia
Published: September 2017

Mutations in GLI3, which encodes a transcription factor of the Hedgehog signaling pathway, cause several developmental anomalies linked to inappropriate tissue patterning. Here, we report a novel missense variant in the fifth zinc finger domain of GLI3 (c.1826G>A; p.(Cys609Tyr)) initially identified in a proband with preaxial polydactyly type IV, developmental delay, sensorineural hearing loss, skeletal, and genitourinary anomalies. Additional family members exhibited various digital anomalies such as preaxial polydactyly, syndactyly, and postaxial polydactyly either in isolation or combined. Functional studies of Cys609Tyr GLI3 in cultured cells showed abnormal GLI3 processing leading to decreased GLI3 repressor production, increased basal transcriptional activity, and submaximal GLI reporter activity with Hedgehog pathway activation, thus demonstrating an intriguing molecular mechanism for this GLI3‐related phenotype. Given the complexity of GLI3 post‐translational processing and opposing biological functions as a transcriptional activator and repressor, our findings highlight the importance of performing functional studies of presumed GLI3 variants. This family also demonstrates how GLI3 variants are variably expressed.
Congenital heart defects in molecularly proven Kabuki syndrome patients
Authors: Digilio Maria Cristina , Gnazzo Maria , Lepri Francesca , Dentici Maria Lisa , Pisaneschi Elisa , Baban Anwar , Passarelli Chiara , Capolino Rossella , Angioni Adriano , Novelli Antonio , Marino Bruno , Dallapiccola Bruno
Published: September 2017

The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left‐sided obstructive lesions, including multiple left‐sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left‐sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation.
FOXP1 haploinsufficiency: Phenotypes beyond behavior and intellectual disability?
Authors: Myers Angela , du Souich Christèle , Yang Connie L. , Borovik Lior , Mwenifumbo Jill , Rupps Rosemarie , Study CAUSES , Lehman Anna , Boerkoel Cornelius F.
Published: September 2017

The forkhead box (FOX) transcription factors have roles in development, carcinogenesis, metabolism, and immunity. In humans FOXP1 mutations have been associated with language and speech defects, intellectual disability, autism spectrum disorder, facial dysmorphisms, and congenital anomalies of the kidney and urinary tract. In mice, Foxp1 plays critical roles in development of the spinal motor neurons, lymphocytes, cardiomyocytes, foregut, and skeleton. We hypothesized therefore that mutations of FOXP1 affect additional tissues in some humans. Supporting this hypothesis, we describe two individuals with novel variants of FOXP1 (NM_032682.5:c.975‐2A>C and NM_032682.5:c.1574G>A) and additional features. One had a lung disease resembling neuroendocrine cell hyperplasia of infancy (NEHI), and the second had a skeletal disorder with undertubulation of the long bones and relapsing‐remitting fevers associated with flushing and edema. Although attribution of these traits to mutation of FOXP1 requires ascertainment of additional patients, we hypothesize that the variable expression of these additional features might arise by means of stochastic developmental variation.
Variable phenotypic expression in a large Noonan syndrome family segregating a novel SOS1 mutation
Authors: van Trier Dorothée C. , Rinne Tuula , Noordam Kees , Draaisma Jos M. , van der Burgt Ineke
Published: September 2017

Noonan syndrome (NS) is an autosomal dominant multisystem condition with a variable phenotype. The most characteristic features are short stature, congenital heart defects, and recognizable facial features. Mutations in SOS1 are found in 10–20% of patients with NS. Different genotype–phenotype studies mention correlations between SOS1 mutations and some features, such as ectodermal abnormalities and specific facial features. We present a large NS family with a novel pathogenic mutation; SOS1 c.3134C>G, p.Pro1045Arg. Ten family members with NS are included with genetically confirmed mutation and clinical evaluation. The phenotype shows a broad spectrum from only few suggestive features for NS in the older generation to typical features in the youngest generation. We report on a novel pathogenic mutation in the SOS1 gene and a large clinical spectrum in a NS family with ten genetically confirmed affected individuals.
A female Viking warrior confirmed by genomics
Authors: Hedenstierna‐Jonson Charlotte , Kjellström Anna , Zachrisson Torun , Krzewińska Maja , Sobrado Veronica , Price Neil , Günther Torsten , Jakobsson Mattias , Götherström Anders , Storå Jan
Published: September 2017

Objectives

The objective of this study has been to confirm the sex and the affinity of an individual buried in a well‐furnished warrior grave (Bj 581) in the Viking Age town of Birka, Sweden. Previously, based on the material and historical records, the male sex has been associated with the gender of the warrior and such was the case with Bj 581. An earlier osteological classification of the individual as female was considered controversial in a historical and archaeological context. A genomic confirmation of the biological sex of the individual was considered necessary to solve the issue.

Materials and methods

Genome‐wide sequence data was generated in order to confirm the biological sex, to support skeletal integrity, and to investigate the genetic relationship of the individual to ancient individuals as well as modern‐day groups. Additionally, a strontium isotope analysis was conducted to highlight the mobility of the individual.

Results

The genomic results revealed the lack of a Y‐chromosome and thus a female biological sex, and the mtDNA analyses support a single‐individual origin of sampled elements. The genetic affinity is close to present‐day North Europeans, and within Sweden to the southern and south‐central region. Nevertheless, the Sr values are not conclusive as to whether she was of local or nonlocal origin.

Discussion

The identification of a female Viking warrior provides a unique insight into the Viking society, social constructions, and exceptions to the norm in the Viking time‐period. The results call for caution against generalizations regarding social orders in past societies.
Transition‐Metal‐Free Ring‐Opening Silylation of Indoles and Benzofurans with (Diphenyl‐tert‐butylsilyl)lithium
Authors: Xu Pan , Würthwein Ernst‐Ulrich , Daniliuc Constantin G. , Studer Armido
Published: September 2017

A practical method is presented for ring opening various indoles and benzofurans with concomitant stereoselective silylation using readily generated (diphenyl‐tert‐butylsilyl)lithium to afford ortho‐β‐silylvinylanilines or ‐phenols. Dearomatization of the heteroarene core proceeds in the absence of any transition‐metal catalyst through addition of a silyl anion and a subsequent stereoselective β‐elimination. DFT calculations provide insight into the mechanism. Functionalizing C−X bond cleavage of heteroarenes is rare and generally requires transition‐metal catalysts.
Vacancy‐Rich Monolayer BiO2−x as a Highly Efficient UV, Visible, and Near‐Infrared Responsive Photocatalyst
Authors: Li Jun , Wu Xiaoyong , Pan Wenfeng , Zhang Gaoke , Chen Hong
Published: September 2017

Vacancy‐rich layered materials with good electron‐transfer property are of great interest. Herein, a full‐spectrum responsive vacancy‐rich monolayer BiO2−x has been synthesized. The increased density of states at the conduction band (CB) minimum in the monolayer BiO2−x is responsible for the enhanced photon response and photo‐absorption, which were confirmed by UV/Vis‐NIR diffuse reflectance spectra (DRS) and photocurrent measurements. Compared to bulk BiO2−x, monolayer BiO2−x has exhibited enhanced photocatalytic performance for rhodamine B and phenol removal under UV, visible, and near‐infrared light (NIR) irradiation, which can be attributed to the vacancy VBi‐O′′′ as confirmed by the positron annihilation spectra. The presence of VBi‐O′′′ defects in monolayer BiO2−x promoted the separation of electrons and holes. This finding provides an atomic level understanding for developing highly efficient UV, visible, and NIR light responsive photocatalysts.
Synthesis and characterization of novel natural rubber based cationic waterborne polyurethane—Effect of emulsifier and diol class chain extender
Authors: Sukhawipat Nathapong , Saetung Nitinart , Pilard Jean‐Francois , Bistac Sophie , Saetung Anuwat
Published: September 2017

Novel cationic waterborne polyurethanes (cWPU) were synthesized by step‐growth polymerization of hydroxyl telechelic natural rubber, molecular weight of 2800 g mol−1, toluene‐2,4‐diisocyanate, N‐methyl diethanolamine (NMDEA) as an emulsifier. The chemical structure of cWPU was confirmed by 1H‐nuclear magnetic resonance and Fourier transform infrared spectroscopy. The amounts of NMDEA and ethylene glycol under isocyanate (NCO) index of 100 on the properties of cWPU were studied. It was found that cWPU was stable under the concentration of NMDEA more than 1.50 mol and the particle sizes decreased with increasing of NMDEA content. Also, contact angle shows more hydrophilic materials by increasing of NMDEA. Extended cWPU was found in two ranges of nano size. Chain extender has strongly affected cWPU film formation, increasing of mechanical properties, and thermal properties. In addition, stress–strain curve and scanning electron microscopy image shows the change of behavior from soft elastic to ductile plastic by adding ethylene glycol. © 2017 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2018, 135, 45715.
In Situ Fabrication of Inorganic/Organic Hybrid Based on Cu/Co‐ZIF and Cu2O
Authors: Wang Tianlong , Ma Hong , Gao Jin , Wang Xinhong , Sun Yuxia , Luo Yang , Zhang Shujing , Xu Jie
Published: September 2017

We successfully fabricate a well‐defined inorganic/organic hybrid Cu2O@Cu/Co‐ZIF (ZIF=zeolitic imidazolate frameworks) by use of growth of dual‐metal Cu/Co‐ZIF on the obtained Cu2O hollow spheres. The key point of the strategy is coupling the in situ self‐sacrificing template. Cu2O and the coordination of metal ions (Cu+ and Co2+) with 2‐methylimidazole. This new hybrid was characterized by powder X‐ray diffraction, (scanning) transmission electron microscopy, energy‐dispersive spectroscopy mapping, in situ FT‐IR spectroscopy, UV/Vis diffuse reflection spectroscopy, N2 sorption measurements, and electron spin resonance. It was evidenced that Cu/Co‐ZIF nanocrystals have been assembled to continuous shells surrounding the Cu2O cores as well as in the voids between layers and inner pores. Cu2O@Cu/Co‐ZIF exhibits visible light responsiveness and holds potential as narrow band gap semiconductor and visible photocatalyst.
How Incidental Confidence Influences Self‐Interested Behaviors: A Double‐Edged Sword
Authors: Tsai Claire I. , Xie Jia Lin
Published: September 2017

The present research investigates how incidental confidence influences self‐interested behaviors. It is well established that being in a psychological state of lower confidence causes people to experience psychological aversion that they are motivated to reduce. We study the transfer effect of confidence; people strive to compensate for lower confidence in one domain by obtaining higher status in other unrelated domains. Prior research has linked money with status and suggested that money can increase confidence. Building on this research, we proposed and showed in four experiments that lower incidental confidence increased self‐interested behaviors that brought financial gains. Drawing on research on competitive altruism, we also predicted and found that when altruism, rather than money, was seen as the primary source of status, the effect of incidental confidence reversed such that lower incidental confidence decreased self‐interested behaviors. Data ruled out alternative explanations and provided consistent evidence for the proposed compensatory mechanism. We also discussed theoretical and practical implications of the present research. Copyright © 2017 John Wiley & Sons, Ltd.