bluuebunny/crossref_metadata_2025_split huggingface dataset:
BRPF1‐associated intellectual disability, ptosis, and facial dysmorphism in a multiplex family
Authors: Pode‐Shakked Naomi , Barel Ortal , Pode‐Shakked Ben , Eliyahu Aviva , Singer Amihood , Nayshool Omri , Kol Nitzan , Raas‐Rothschild Annick , Pras Elon , Shohat Mordechai 
Published: April 2019

AbstractBackgroundOver 500 epigenetic regulators have been identified throughout the human genome. Of these, approximately 30 chromatin modifiers have been implicated thus far in human disease. Recently, variants in BRPF1, encoding a chromatin reader, have been associated with a previously unrecognized autosomal dominant syndrome manifesting with intellectual disability (ID), hypotonia, dysmorphic facial features, ptosis, and/or blepharophimosis in 22 individuals.Patients and MethodsWe report a multiply affected nonconsanguineous family of mixed Jewish descent who presented due to ID in three male siblings. Molecular analysis of the family was pursued using whole exome sequencing (WES) and subsequent Sanger sequencing.ResultsWhole exome sequencing analysis brought to the identification of a novel heterozygous truncating mutation (c.556C>T, p.Q186*) in the BRPF1 gene in the affected siblings and their mother. The four affected individuals showed varying degrees of intellectual disability, distinct facial features including downslanted palpebral fissures, ptosis, and/or blepharophimosis. Their clinical characteristics are discussed in the context of previously reported patients with the BRPF1‐related phenotype.ConclusionThe reported family contributes to the current knowledge regarding this unique and newly recognized genetic disorder, and further implicates the role of BRPF1 in human brain development.
The Kamlet‐Jacobs Parameter φ: A Measure of Intrinsic Detonation Potential
Authors: Politzer Peter , Murray Jane S. 
Published: April 2019

AbstractIt was shown by Kamlet and Jacobs that an explosive's detonation velocity D and detonation pressure PD are governed largely by (a) the loading density of the explosive, and (b) a factor φ that depends upon the number and masses of gaseous detonation products and the accompanying heat release. For a series of different explosives, we show that the density and φ are both important in determining D and PD. For a given explosive, however, φ is approximately constant and so D and PD correlate quite well with the density, as has long been known. We propose that φ be interpreted as a measure of the intrinsic detonation potential of an explosive, that is independent of the external factors that affect the loading density. Comparison of φ values for different explosives can provide useful insights into features that promote high detonation velocities and detonation pressures. The parameter φ is related to a property introduced earlier, explosive power, but φ takes into account the fact that part of the carbon content of an explosive typically remains as a residue after the detonation process.
Activation-Induced Cytidine Deaminase Regulates Fibroblast Growth Factor/Extracellular Signal-Regulated Kinases Signaling to Achieve the Naïve Pluripotent State During Reprogramming
Authors: Kumar Ritu , Evans Todd 
Published: April 2019

Abstract
Induced pluripotent stem cells (iPSCs) derived by in vitro reprogramming of somatic cells retain the capacity to self-renew and to differentiate into many cell types. Pluripotency encompasses multiple states, with naïve iPSCs considered as ground state, possessing high levels of self-renewal capacity and maximum potential without lineage restriction. We showed previously that activation-induced cytidine deaminase (AICDA) facilitates stabilization of pluripotency during reprogramming. Here, we report that Acida−/− iPSCs, even when successfully reprogrammed, fail to achieve the naïve pluripotent state and remain primed for differentiation because of a failure to suppress fibroblast growth factor (FGF)/extracellular signal-regulated kinases (ERK) signaling. Although the mutant cells display marked genomic hypermethylation, suppression of FGF/ERK signaling by AICDA is independent of deaminase activity. Thus, our study identifies AICDA as a novel regulator of naïve pluripotency through its activity on FGF/ERK signaling. Stem Cells 2019;37:1003–1017

Significance Statement Growth factor signaling requirements that modulate pluripotent state are well studied. However, the epigenetic basis of how the dynamic state of pluripotent cells is regulated and stabilized is largely a black box. The current study is important because the results show that AICDA is essential for reprogramming to ground state. A better understanding for how to stabilize ground state pluripotent cells is of fundamental importance for the use of pluripotent cell sources in disease modeling and potential cellular therapies.
Research and application of a new super‐long span roof based on cable structure
Authors: Li Yu , Yang Li 
Published: April 2019

SummaryWith the continuous development of urban processes, people's demand for large‐span space is getting stronger and stronger, and the application of large‐span building structures is becoming more and more extensive. This paper introduces a new type of suspension cable roof that uses the building's own weight to increase the tension of the cable to achieve a super‐long span. The rationality of the structural system is analyzed, and the dynamic performance, wind resistance, and seismic performance of the structure are analyzed with a simple example. At the same time, measures to further improve the structural span are proposed. Finally, it provides the forms of building that are feasible in this structural form, which provides reference and guidance for the design and construction of long‐span buildings, which is of great significance.
A computer‐assisted method for the diblock copolymer model
Authors: Cai Shuting , Watanabe Yoshitaka 
Published: April 2019

AbstractWe propose a computer‐assisted method by which to enclose a solution of the diblock copolymer model. We begin by using the Newton method to obtain a solution, and we then prove that the residual part of the approximation is a fixed point of a compact operator. Next, using a computer, we construct a set which satisfies the hypothesis of the Banach fixed‐point theorem for the operator in a certain Sobolev space, which therefore contains a unique solution. Finally, we present some verified results.
The opportunism of populists and the defense of constitutional liberalism
Authors: Scheppele Kim Lane 
Published: April 2019

AbstractLiberal constitutionalism is under attack from a new breed of autocrats broadly classified as populist. These populists understand the weaknesses of constitutional liberalism and attack their opponents with criticisms that take advantage of internal weaknesses of the theory. But a closer analysis of theoretical framework used by populists to substitute for constitutional liberalism reveals that they are not really committed to populism in any serious sense. Instead, they have abandoned liberalism in the quest for raw power. Focusing on Viktor Orbán of Hungary and his chief ideologist András Lánczi, this article shows how their public critique of liberalism has attempted to wrong-foot their critics and how their recipe for gaining and wielding political power is only populist to the extent that these leaders are determined to (and often succeed in) winning elections. By peeling back the cover of populist ideology to look at the theories of legitimation under which they rule, however, we can see that the new breed of autocrats aims at primarily constitutional deconstruction through the concentration of political power in one leader. This sort of challenge to liberal constitutionalism is easily countered.
Varieties of populism
Authors: Tushnet Mark 
Published: April 2019

AbstractContemporary discussions of populism elide important distinctions between the ways in which populist leaders and movements respond to the failures of elites to follow through on the promises associated with international social welfare constitutionalism. After laying out the political economy of populisms’ origins, this Article describes the relation between populisms and varieties of liberalism, and specifically the relation between populisms and judicial independence understood as a “veto point” occupied by the elites that populists challenge. It then distinguishes left-wing populisms’ acceptance of the social welfare commitments of late twentieth century liberalism and its rejection of some settled constitutional arrangements that, in populists’ views, obstruct the accomplishment of those commitments. It concludes with a description of the core ethnonationalism of right-wing populism, which sometimes contingently appears in left-wing populisms but is not one the latter’s core components.
Introduction—Public law and populism
Authors: Dixon Rosalind 
Published: April 2019

AbstractIn an age of profound democratic anxiety, significant academic attention has been paid to the crisis in constitutional democracy. Constitutional lawyers, however, are still grappling with the relationship between public law and the current actual and perceived threats facing constitutional democracy in countries worldwide. This Introduction considers how the articles in this special volume address three pressing questions. How should we should define the current threats to democracy, and populist challenge? Second, how might public law be a potential cause or contributing factor? Third, how might public law still provide some answers to the contemporary challenges to constitutional democracy? In Prof Dixon’s view, constitutional democracy is a good worth preserving and there are models of at least relative “success” in the current constitutional universe. But Prof Dixon shares the view of many contributors to the special volume that the challenge is immense, and urgent, and that there are no easy solutions.
PRACTICE IS IMPORTANT BUT HOW ABOUT ITS QUALITY?
Authors: Sato Masatoshi , McDonough Kim 
Published: April 2019

AbstractThis study explored the impact of contextualized practice on second language (L2) learners’ production of wh-questions in the L2 classroom. It examined the quality of practice (correct vs. incorrect production) and the contribution of declarative knowledge to proceduralization. Thirty-four university-level English as a foreign language learners first completed a declarative knowledge test. Then, they engaged in various communicative activities over five weeks. Their production of wh-questions was coded for accuracy (absence of errors) and fluency (speech rate, mean length of pauses, and repair phenomena). Improvement was measured as the difference between the first and last practice sessions. The results showed that accuracy, speech rate, and pauses improved but with distinct patterns. Regression models showed that declarative knowledge did not predict accuracy or fluency; however, declarative knowledge assisted the learners to engage in targetlike behaviors at the initial stage of proceduralization. Furthermore, whereas production of accurate wh-questions predicted accuracy improvement, it had no impact on fluency.
Pour une histoire globale du monde atlantique ou des histoires connectées dans et au-delà du monde atlantique?
Authors: Vidal Cécile 
Published: April 2019

RésuméL’article porte sur l’histoire atlantique qui a pris son essor en Amérique du Nord à partir des années 1990. À partir d’une lecture critique de deux ouvrages majeurs, récemment parus, concernant ce « nouveau » courant historiographique, il expose les multiples débats qui divisent la communauté des atlanticistes quant à la manière de conceptualiser le monde atlantique, de pratiquer l’histoire atlantique et d’envisager le devenir des études atlantiques. Il défend l’idée que le monde atlantique doit demeurer un cadre d’analyse et ne pas devenir l’objet même d’étude. L’objectif est d’écrire de la sorte une histoire située qui, tout en tenant compte du point de vue de tous les acteurs concernés, mette l’accent sur la redéfinition et la renégociation des enjeux et des rapports de pouvoir entre les individus, les groupes et les formations sociales et politiques dans ce monde interconnecté sous l’effet du projet colonialiste et impérialiste des Européens.
Longitudinal associations of callous-unemotional and oppositional defiant behaviors over a three-year interval for Spanish children
Authors: Servera Mateu , Seijas Raquel , García-Banda Gloria , Barry Christopher T. , Beauchaine Theodore P. , Burns G. Leonard 
Published: April 2019

AbstractThe objective was to determine the longitudinal associations between callous-unemotional (CU) and oppositional defiant (OD) behaviors from the first to fourth grades for Spanish children. Four possible outcomes were evaluated: (a) CU behaviors in the first grade predict increases in OD behaviors in the fourth grade, controlling for OD behaviors in the first grade; (b) OD behaviors in the first grade predict increases in CU behaviors in the fourth grade, controlling for CU behaviors in the first grade; (c) both unique effects are significant; and (d) neither unique effect is significant. A longitudinal panel model with two latent variables (CU and OD behaviors), three sources (mothers, fathers, teachers), and two occasions (spring of the first and fourth grades) was used to evaluate the four possibilities among 758 (54% boys) first grade and 469 (53% boys) fourth grade Spanish children. For mother-, father-, and teacher-reports, OD behaviors in the first grade predicted increases in CU behaviors in the fourth grade, after controlling for CU behaviors in the first grade, whereas CU behaviors in the first grade did not predict increases in OD behaviors in the fourth grade, after controlling for OD behaviors in the first grade. OD behaviors thus conferred independent vulnerability to increases in CU behaviors 3 years later among young children.
Semantic Memory Activation After Acute Exercise in Healthy Older Adults
Authors: Won Junyeon , Alfini Alfonso J. , Weiss Lauren R. , Michelson Corey S. , Callow Daniel D. , Ranadive Sushant M. , Gentili Rodolphe J. , Smith J. Carson 
Published: April 2019

AbstractObjectives:A growing body of research suggests that regular participation in long-term exercise is associated with enhanced cognitive function. However, less is known about the beneficial effects of acute exercise on semantic memory. This study investigated brain activation during a semantic memory task after a single session of exercise in healthy older adults using functional magnetic resonance imaging (fMRI).Methods:Using a within-subjects counterbalanced design, 26 participants (ages, 55–85 years) underwent two experimental visits on separate days. During each visit, participants engaged in 30 min of rest or stationary cycling exercise immediately before performing a Famous and Non-Famous name discrimination task during fMRI scanning.Results:Acute exercise was associated with significantly greater semantic memory activation (Famous>Non-Famous) in the middle frontal, inferior temporal, middle temporal, and fusiform gyri. A planned comparison additionally showed significantly greater activation in the bilateral hippocampus after exercise compared to rest. These effects were confined to correct trials, and as expected, there were no differences between conditions in response time or accuracy.Conclusions:Greater brain activation following a single session of exercise suggests that exercise may increase neural processes underlying semantic memory activation in healthy older adults. These effects were localized to the known semantic memory network, and thus do not appear to reflect a general or widespread increase in brain blood flow. Coupled with our prior exercise training effects on semantic memory-related activation, these data suggest the acute increase in neural activation after exercise may provide a stimulus for adaptation over repeated exercise sessions. (JINS, 2019,25, 557–568)
Improving Compositional Accuracy in APT Analysis of Carbides Using a Decreased Detection Efficiency
Authors: Thuvander Mattias , Shinde Deodatta , Rehan Arbab , Ejnermark Sebastian , Stiller Krystyna 
Published: April 2019

AbstractThe composition of carbides in steel, measured by atom probe tomography, can be influenced by limitations in the ion detector system. When carbides are analyzed, many ions tend to field evaporate from the same region of the specimen during the same laser or voltage pulse. This results in a so-called multiple event, meaning that several ions impact the detector in close proximity both in time and space. Due to a finite detector dead-time not all ions can be detected, a phenomenon known as detector pile-up. The evaporation behavior of carbon is often different than the evaporation behavior of metals when analyzing alloy carbides, leading to preferential loss of carbon ions, and a measured carbon concentration below the expected value. This effect becomes stronger as the overall detection efficiency gets higher. Here, the detection efficiency was deliberately reduced by inserting a grid into the flight-path, which resulted in a higher and more correct carbon concentration, accompanied by an increase in the statistical uncertainty.
Trends in management and outcomes of vascular emergencies in the	nationwide inpatient sample
Authors: Bath Jonathan , Hartwig Jacob , Dombrovskiy Viktor Y. , Vogel Todd R. 
Published: April 2019

Summary: Background: To evaluate trends in frequency, mortality and treatment for non-traumatic vascular emergencies (VE) in the US. Methods: VE in the Nationwide Inpatient Sample (2005–2014) were identified. ICD-9 CM diagnosis and procedures codes captured six common VE. Results: 228,210,504 emergency admissions with 317,396 procedures for VE were estimated. Mean age was 67.8 years and were primarily men (56.1 %; p < 0.0001). The commonest VE was Acute Limb Ischemia (ALI) (82.4 %) followed by ruptured AAA (10.8 %) and Acute Mesenteric Ischemia (4.71 %). VE increased from 132.8 per 100,000 admissions in 2005 to 153.6 in 2014 (p < 0.001), with mortality decrease for all VE (13.8 % vs. 9.1 %; p < 0.0001). Length of stay decreased (median 8 vs. 7 days; p < 0.0001) but cost of care increased (median $ 25,443 vs. $ 29,353; p < 0.0001). Endovascular treatment increased overall for VE from 23.7 % in 2005 to 37.2 % in 2014 (p < 0.0001). Hospital mortality for VE decreased overall, except ruptured thoracoabdominal aortic aneurysm with mortality decrease with endovascular treatment (34.3 vs. 11.1; p = 0.04) and mortality increase with open treatment (44.7 vs. 47.6; p = 0.06). ALI overall mortality decreased from 8.1 % to 5.7 % (p < 0.0001) due to reduced open surgical mortality from 9.6 % to 7.4 % (p < 0.0001); endovascular mortality did not improve over time (4.0 % vs. 3.4 %; p = 0.45). Hospital mortality also increased for endovascular treatment of ruptured thoracic aortic aneurysm (rTAA) from 14.9 % to 27.4 % (p = 0.0003) during this period. Conclusions: VE frequency increased with a decrease in overall mortality over time. Overall hospital stay has decreased but with an increase in the cost of care. Open surgical mortality for VE has also decreased overall, suggesting perioperative care improvements, with the exception of ruptured thoracoabdominal aortic aneurysm. Endovascular utilization for VE has significantly increased; associated with lower mortality for most VE, although an increase in hospital mortality after endovascular repair of rTAA was seen. This may be due to an increased implementation of endovascular repair for patients not previously eligible for surgery due to high risk. We recommend careful selection of patients for rTAA treatment as mortality has increased despite endovascular therapy and at an increased cost of care.
Shear Velocity Structure Beneath Saudi Arabia From the Joint Inversion of P and S Wave Receiver Functions, and Rayleigh Wave Group Velocity Dispersion Data
Authors: Tang Zheng , Mai P. Martin , Julià Jordi , Zahran Hani 
Published: April 2019

AbstractWe develop a new 3‐D shear velocity model for the lithosphere and sublithospheric mantle under Saudi Arabia by jointly inverting P wave receiver functions, S wave receiver functions, and fundamental‐mode Rayleigh wave group velocities. P and S receiver functions are calculated from earthquakes recorded between 2012 and 2015 at 156 Saudi National Seismic Network stations operated by the Saudi Geological Survey. Rayleigh wave dispersion data are extracted from independent tomographic studies. Our model reveals significant lateral variations in crustal and upper‐mantle S velocity below Saudi Arabia. Particularly, a low‐velocity zone, with minimum S velocity of ~4.0 km/s in the depth range of 70‐190 km, is observed under the Arabian Shield coinciding with Cenozoic surface volcanism. The low‐velocity zone is found consistent with the presence of partial melts in the mantle and interpreted as a potential deep magma source for the volcanism in western Arabia. We propose that magmas responsible for the Arabian volcanism may be derived from multiple sources. Both lithospheric thinning and the Afar plume trigger magma production beneath southwestern Arabia, while decompression melting caused by the lithospheric thinning may be the main factor in the central and northern portions. Furthermore, we find evidence for localized crustal low shear velocity anomalies (2–4% reductions) that appear spatially correlated to the volcanism; these may be due to fractures caused by magma ascent and small amounts of partial melt in the crust. This spatial distribution of S velocity reductions may indicate the plumbing system for magma ascent underneath western Arabia.
First‐Principles Study of FeO2Hx Solid and Melt System at High Pressures: Implications for Ultralow‐Velocity Zones
Authors: Deng Jie , Karki Bijaya B. , Ghosh Dipta B. , Lee Kanani K. M. 
Published: April 2019

AbstractPyrite‐type FeO2Hx (P phase) has recently been suggested as a possible alternative to explain ultralow‐velocity zones due to its low seismic velocity and high density. Here we report the results on the congruent melting temperature and melt properties of P phase at high pressures from first‐principles molecular dynamics simulations. The results show that P phase would likely be melted near the core–mantle boundary. Liquid FeO2Hx has smaller density and smaller bulk sound velocity compared to the isochemical P phase. As such, relatively small amounts of liquid FeO2Hx could account for the observed seismic anomaly of ultralow‐velocity zones. However, to maintain the liquid FeO2Hx within the ultralow‐velocity zones against compaction requires special physical conditions, such as relatively high viscosity of the solid matrix and/or vigorous convection of the overlying mantle.