bluuebunny/crossref_metadata_2025 huggingface dataset:
Technology and the New Economy
Authors: Bai Chong-En , Yuen Chi-Wa
Published: December 2018

Essays on the effects of information technology on the economy.

One of the most important forces driving economic performance in the United States and other countries during the 1990s was the rise of information technology. The new technology has had such a significant impact on the economy that "the new economy" emerged as a popular term in both the media and academia.

This book, written in an accessible style, examines basic questions about the effects of information technology on various aspects of the economy. The topics include the relationship between innovation and the stock market value of the innovating firm; competition policy; demand factors as determinants of growth; institutional aspects of the innovation process; and the effectiveness of monetary policy in stabilizing the economy.
Veritas
Authors: Vision Gerald
Published: December 2018

A restatement of the correspondence theory of truth together with a defense against objections and alternative theories, including deflationism, minimalism, and pluralism.

In Veritas, Gerald Vision defends the correspondence theory of truth—the theory that truth has a direct relationship to reality—against recent attacks, and critically examines its most influential alternatives. The correspondence theory, if successful, explains one way in which we are cognitively connected to the world; thus, it is claimed, truth—while relevant to semantics, epistemology, and other studies—also has significant metaphysical consequences. Although the correspondence theory is widely held today, Vision points to an emerging orthodoxy in philosophy that claims that truth as such carries no significant weight in philosophical explanations. He devotes much of the book to a criticism of that outlook and to a less vulnerable formulation of the correspondence theory.

Vision defends the correspondence theory by both presenting evidence for correspondence and examining the claims made by such alternative theories as deflationism, minimalism, and pluralism. The techniques of the argument are thoroughly analytic, but the problem confronted is broadly humanistic. The question examined—how we, as thinking beings, are connected to and manage to cope in a world that was not designed for our comfort or convenience—is more likely to be raised by continentalists, but is approached here with the tools of clarity and precision more highly prized in analytic philosophy. The book seeks to avoid both the obscurantism that infects much continental thought and the overly technical concerns and methodology that limit the interest of much work in analytic philosophy. It thus provides a rigorous but largely nontechnical treatment of the topic that will be of interest not only to readers familiar with philosophy but also to those with a background in literary theory and linguistics.

Bradford Books imprint
Visual Versions
Authors: Schwartz Robert
Published: December 2018

These essays by Robert Schwartz on topics in the theory of vision are written from a pragmatic perspective. The issues and arguments will interest both philosophers and psychologists, covering new ground and bridging gaps between these disciplines. Schwartz begins historically, with discussions of problems raised and solutions offered in Bishop Berkeley's writings on vision, presenting Berkeley's views on spatial perception and the qualitative aspects of sensory experience in the context of recent theoretical and empirical work in vision theory. Schwartz then turns to debates in both the philosophical and psychological literature over the view that perception is inferential and thus "indirect." Critically surveying competing characterizations of the idea of "inferential processes" he argues the need either to reframe radically the question or drop the issue. Next, Schwartz discusses pictorial representation and research on picture perception. Drawing on the work of Nelson Goodman, Schwartz explains and defends the advantages of a symbolic approach to both topics. Finally, he examines the quagmires that often develop when metaphysical concerns about the "real" and our ability to perceive it infect discussions and claims in the theory of vision. After analyzing issues arising in current psychological research on "object" perception, Schwartz turns to debates over the supposed essential nature of colors. An appreciation of the empirical and theoretical work on color perception suggests that there is no single or privileged analysis of the notion of "real colors." Schwartz circles back in the end to what he calls "that old chestnut of the philosophy of perception"—controversies over "the objects of perception"—and takes an Austinian look at the topic.

Bradford Books imprint
Water, Race, and Disease
Authors: Troesken Werner
Published: December 2018

A qualitative and quantitative analysis of the effect of public water and sewer systems on African American life expectancy in the Jim Crow era.

Why, at the peak of the Jim Crow era early in the twentieth century, did life expectancy for African Americans rise dramatically? And why, when public officials were denying African Americans access to many other public services, did public water and sewer service for African Americans improve and expand? Using the qualitative and quantitative tools of demography, economics, geography, history, law, and medicine, Werner Troesken shows that the answers to these questions are closely connected. Arguing that in this case, racism led public officials not to deny services but to improve them—the only way to "protect" white neighborhoods against waste from black neighborhoods was to install water and sewer systems in both—Troesken shows that when cities and towns had working water and sewer systems, typhoid and other waterborne diseases were virtually eradicated. This contributed to the great improvements in life expectancy (both in absolute terms and relative to whites) among urban blacks between 1900 and 1940. Citing recent demographic and medical research findings that early exposure to typhoid increases the probability of heart problems later in life, Troesken argues that building water and sewer systems not only reduced waterborne disease rates, it also improved overall health and reduced mortality from other diseases. Troesken draws on many independent sources of evidence, including data from the Negro Mortality Project, econometric analysis of waterborne disease rates in blacks and whites, analysis of case law on discrimination in the provision of municipal services, and maps showing the location of black and white households. He argues that all evidence points to one conclusion: that there was much less discrimination in the provision of public water and sewer systems than would seem likely in the era of Jim Crow.
The Radiance of France
Authors: Hecht Gabrielle
Published: December 2018

How it happened that technological prowess and national glory (or “radiance,” which also means “radiation” in French) became synonymous in France as nowhere else.

In the aftermath of World War II, as France sought a distinctive role for itself in the modern, postcolonial world, the nation and its leaders enthusiastically embraced large technological projects in general and nuclear power in particular. The Radiance of France asks how it happened that technological prowess and national glory (or “radiance,” which also means “radiation” in French) became synonymous in France as nowhere else.

To answer this question, Gabrielle Hecht has forged an innovative combination of technology studies and cultural and political history in a book that, as Michel Callon writes in the new foreword to this edition, “not only sheds new light on the role of technology in the construction of national identities” but is also “a seminal contribution to the history of contemporary France.” Proposing the concept of technopolitical regime as a way to analyze the social, political, cultural, and technological dynamics among engineering elites, unionized workers, and rural communities, Hecht shows how the history of France's first generation of nuclear reactors is also a history of the multiple meanings of nationalism, from the postwar period (and France's desire for post-Vichy redemption) to 1969 and the adoption of a “Frenchified” American design.

This paperback edition of Hecht's groundbreaking book includes both Callon's foreword and an afterword by the author in which she brings the story up to date, and reflects on such recent developments as the 2007 French presidential election, the promotion of nuclear power as the solution to climate change, and France's aggressive exporting of nuclear technology.
Lenin's Laureate
Authors: Josephson Paul R.
Published: December 2018

The life and work of a leading Soviet physicist and an exploration of the strengths and weaknesses of Soviet science from Stalin through Gorbachev.

In 2000, Russian scientist Zhores Alferov shared the Nobel Prize for Physics for his discovery of the heterojunction, a semiconductor device the practical applications of which include LEDs, rapid transistors, and the microchip. The Prize was the culmination of a career in Soviet science that spanned the eras of Stalin, Khrushchev, and Gorbachev—and continues today in the postcommunist Russia of Putin and Medvedev.

In Lenin's Laureate, historian Paul Josephson tells the story of Alferov's life and work and examines the bureaucratic, economic, and ideological obstacles to doing state-sponsored scientific research in the Soviet Union. Lenin and the Bolsheviks built strong institutions for scientific research, rectifying years of neglect under the Czars. Later generations of scientists, including Alferov and his colleagues, reaped the benefits, achieving important breakthroughs: the first nuclear reactor for civilian energy, an early fusion device, and, of course, the Sputnik satellite. Josephson's account of Alferov's career reveals the strengths and weaknesses of Soviet science—a schizophrenic environment of cutting-edge research and political interference. Alferov, born into a family of Communist loyalists, joined the party in 1967. He supported Gorbachev's reforms in the 1980s, but later became frustrated by the recession-plagued postcommunist state's failure to fund scientific research adequately. An elected member of the Russian parliament since 1995, he uses his prestige as a Nobel laureate to protect Russian science from further cutbacks.

Drawing on extensive archival research and the author's own discussions with Alferov, Lenin's Laureate offers a unique account of Soviet science, presented against the backdrop of the USSR's turbulent history from the revolution through perestroika.
The Foundations of Cognitive Archaeology
Authors: Abramiuk Marc A.
Published: December 2018

An empirically supported proposal for synthesizing multiple approaches to the study of the mind in the past.

In The Foundations of Cognitive Archaeology, Marc Abramiuk proposes a multidisciplinary basis for the study of the mind in the past, arguing that archaeology and the cognitive sciences have much to offer one another. Abramiuk draws on relevant topics from philosophy, biological anthropology, cognitive psychology, cognitive anthropology, and archaeology to establish theoretically founded and empirically substantiated principles of a discipline that integrates different approaches to mind-related archaeological research.

Abramiuk discusses the two ways that archaeologists have traditionally viewed the human mind: as a universal or as a relative interface with the environment. He argues that neither view by itself can satisfactorily serve as a basis for gleaning insight into all aspects of the mind in the past and, therefore, the mind is more appropriately studied using multiple approaches. He explains the rationale for using these approaches in mind-related archaeological research, reviewing the literature in both cognitive psychology and cognitive anthropology on human memory, perception, and reasoning. Drawing on archaeological and genetic evidence, Abramiuk investigates the evolution of the mind through the Upper Paleolithic era—when the ancient mind became functionally comparable to the modern human mind. Finally, Abramiuk offers a model for the establishment of a discipline dealing with the study of the mind in the past that integrates all the approaches discussed.
Young People, Ethics, and the New Digital Media
Authors: James Carrie , Davis Katie , Flores Andrea , Francis John M. , Pettingill Lindsay , Rundle Margaret , Gardner Howard
Published: December 2018

Social networking, blogging, vlogging, gaming, instant messaging, downloading music and other content, uploading and sharing their own creative work: these activities made possible by the new digital media are rich with opportunities and risks for young people. This report, part of the GoodPlay Project, undertaken by researchers at Harvard Graduate School of Education's Project Zero, investigates the ethical fault lines of such digital pursuits. The authors argue that five key issues are at stake in the new media: identity, privacy, ownership and authorship, credibility, and participation. Drawing on evidence from informant interviews, emerging scholarship on new media, and theoretical insights from psychology, sociology, political science, and cultural studies, the report explores the ways in which youth may be redefining these concepts as they engage with new digital media. The authors propose a model of "good play" that involves the unique affordances of the new digital media; related technical and new media literacies; cognitive and moral development and values; online and offline peer culture; and ethical supports, including the absence or presence of adult mentors and relevant educational curricula. This proposed model for ethical play sets the stage for the next part of the GoodPlay project, an empirical study that will invite young people to share their stories of engagement with the new digital media.

The John D. and Catherine T. MacArthur Foundation Reports on Digital Media and Learning
The Plasmodium knowlesi Pk41 surface protein diversity, natural selection, sub population and geographical clustering: a 6-cysteine protein family member
Authors: Ahmed Md Atique , Chu Ki-Back , Quan Fu-Shi
Published: December 2018

Introduction

The zoonotic malaria parasite Plasmodium knowlesi has currently become the most dominant form of infection in humans in Malaysia and is an emerging infectious disease in most Southeast Asian countries. The P41 is a merozoite surface protein belonging to the 6-cysteine family and is a well-characterized vaccine candidate in P. vivax and P. falciparum; however, no study has been done in the orthologous gene of P. knowlesi. This study investigates the level of polymorphism, haplotypes and natural selection of pk41 genes in clinical isolates from Malaysia.

Method

Thirty-five full-length pk41 sequences from clinical isolates of Malaysia along with four laboratory lines (along with H-strain) were downloaded from public databases. For comparative analysis between species, orthologous P41 genes from P. falciparum, P. vivax, P. coatneyi and P. cynomolgi were also downloaded. Genetic diversity, polymorphism, haplotype and natural selection were determined using DnaSP 5.10 software. Phylogenetic relationships between Pk41 genes were determined using MEGA 5.0 software.

Results

Analysis of 39 full-length pk41 sequences along with the H-strain identified 36 SNPs (20 non-synonymous and 16 synonymous substitutions) resulting in 31 haplotypes. Nucleotide diversity across the full-length gene was low and was similar to its ortholog in P. vivax; pv41. Domain-wise amino acid analysis of the two s48/45 domains indicated low level of polymorphisms for both the domains, and the glutamic acid rich region had extensive size variations. In the central domain, upstream to the glutamate rich region, a unique two to six (K-E)n repeat region was identified within the clinical isolates. Overall, the pk41 genes were indicative of negative/purifying selection due to functional constraints. Domain-wise analysis of the s48/45 domains also indicated purifying selection. However, analysis of Tajima’s D across the genes identified non-synonymous SNPs in the s48/45 domain II with high positive values indicating possible epitope binding regions. All the 6-cysteine residues within the s48/45 domains were conserved within the clinical isolates indicating functional conservation of these regions. Phylogenetic analysis of full-length pk41 genes indicated geographical clustering and identified three subpopulations of P. knowlesi; one originating in the laboratory lines and two originating from Sarawak, Malaysian Borneo.

Conclusion

This is the first study to report on the polymorphism and natural selection of pk41 genes from clinical isolates of Malaysia. The results reveal that there is low level of polymorphism in both s48/45 domains, indicating that this antigen could be a potential vaccine target. However, genetic and molecular immunology studies involving higher number of samples from various parts of Malaysia would be necessary to validate this antigen’s candidacy as a vaccine target for P. knowlesi.
Epistemology and Ontology
Authors: Crookes Graham
Published: December 2018

Epistemology (the nature of knowledge) and ontology (the nature of what is) are important concepts for understanding second language learning. These concepts are explained and applied to what we know in second language contexts, where professional knowledge can be derived from research or experience, for the benefit of second language learners, teachers, and researchers.
Genre and Discourse Analysis in Language for Specific Purposes
Authors: Hyland Ken
Published: December 2018

Language for specific purposes (LSP) refers to a distinctive approach to language education that focuses on the particular linguistic features, discourse practices, and communicative skills used by target groups. Its success depends on accurately identifying what these features and practices are so they can be taught to students, and this has been greatly assisted since the late 1980s by the emergence of genre analysis. Genre analysis has become established as one of the most popular and productive frameworks for the study of specialized communication in academic, professional, and institutional contexts. Essentially, the approach is used to describe texts within textual and social contexts, rejecting the idea that individual texts should be treated in isolation from either their use or other texts. This entry will offer an overview of the importance of genre and discourse analysis in this area of research and pedagogy.
Language Planning: Qin Shihuangdi's Contribution
Authors: Wang Xiaomei
Published: December 2018

This entry introduces the contribution of Qin Shihuangdi of China in the Qin dynasty in the area of language planning. He implemented the Shutongwen policy to unify different varieties of Chinese writing in order to consolidate his governance before and after he unified China. The Small Seal Script, Xiaozhuan, was chosen as the standard code to be used in official domains. In light of the theory of language planning, the choice of the prestigious code Xiaozhuan and the compilation of textbooks are important procedures in corpus planning. Meanwhile, Qin Shihuangdi also allowed the use of Lishu, the Clerical Script, in informal domains, which is another significant contribution in terms of the development of Chinese characters. All in all, Qin Shihuangdi was the first person in China who planned the Chinese script consciously to meet the needs of unification and communication among the people. He established the tradition of using a unified script in China, which was a great contribution to the Chinese culture.
Needs Analysis
Authors: Long Michael H.
Published: December 2018

Learners vary considerably in the uses they have or will have for a new language. Identifying their communicative needs makes it possible to design courses that are visibly relevant for them and that are efficient because they include what they do need, and exclude what they do not. For several reasons, a task‐based needs analysis is more appropriate than a grammatically based one. It has two parts. First, target tasks are identified, triangulating results obtained from a variety of sources of information, of which domain experts are the most important, using a variety of methods. Then, genuine samples of spoken and written target discourse surrounding successful target task accomplishment are analyzed, and prototypical models developed. The L2 input the models provide is elaborated, not simplified, and suitable for teaching materials in the form of series of pedagogic tasks.
Roles for Corrective Feedback in Second Language Instruction
Authors: Lyster Roy
Published: December 2018

This entry addresses the roles of oral corrective feedback in classroom settings in terms of its various types, functions, and effects. It concludes with suggestions for future classroom‐based research on corrective feedback.
Interval observers for linear functions of state vectors of linear fractional‐order systems with delayed input and delayed output
Authors: Huong Dinh Cong
Published: December 2018

This paper addresses the problem of interval observer design for linear functions of state vectors of linear fractional‐order systems, which are subjected to time delays in the measured output as well as the control input. By using the information of both the delayed output and input, we design two linear functional state observers to compute two estimates, an upper one and a lower one, which bound the unmeasured linear functions of state vectors. As a particular case with output delay only, we design a linear functional state observer to estimate (asymptotically) the unmeasured linear functions of state vectors. Existence conditions of such observers are provided, and some of them are translated into a linear programming problem, in which the observers' matrices can be effectively computed. Constructive design algorithms are introduced. Numerical examples are provided to illustrate the design procedure, practicality, and effectiveness of the proposed design method.
Extracellular Vesicle–Mimetic Ghost Nanovesicles for Delivering Anti‐Inflammatory Drugs to Mitigate Gram‐Negative Bacterial Outer Membrane Vesicle–Induced Systemic Inflammatory Response Syndrome
Authors: Go Gyeongyun , Lee Jaewook , Choi Dong‐Sic , Kim Sang Soo , Gho Yong Song
Published: December 2018

Sepsis is one of the major causes of death in hospital patients and is represented by systemic inflammatory response syndrome (SIRS) associated with infection. Gram‐negative bacteria including Escherichia coli can provoke sepsis by stimulating the immune systems. Outer membrane vesicles (OMVs), nanosized vesicular structures derived from Gram‐negative bacteria, contain several pathogen‐associated molecular patterns, and are demonstrated to mediate SIRS. Here, extracellular vesicle–mimetic ghost nanovesicles loaded with dexamethasone, an anti‐inflammatory drug, are developed using alkaline solution, sonication, and buoyant density gradient ultracentrifugation. These ghost nanovesicles have comparable physical features with naturally released extracellular vesicles but have 200‐fold higher production yields than extracellular vesicles. Importantly, these ghost nanovesicles are devoid of potentially unwanted luminal cargos, including cytosolic proteins and nucleic acids. By maintaining the same topology as the parental cells, these dexamethasone‐loaded ghost nanovesicles derived from human U937 monocytes reduce the release of interleukin‐8 from OMV‐treated endothelial cells in vitro, and mitigate the symptoms of OMV‐induced SIRS in vivo. This study sheds light on using extracellular vesicle–mimetic ghost nanovesicles to deliver therapeutics to treat diseases such as bacterial sepsis.
Poly(β‐Amino Esters): Synthesis, Formulations, and Their Biomedical Applications
Authors: Liu Yong , Li Yuanfeng , Keskin Damla , Shi Linqi
Published: December 2018

Poly(β‐amino ester) (abbreviated as PBAE or PAE) refers to a polymer synthesized from an acrylate and an amine by Michael addition and has properties inherent to tertiary amines and esters, such as pH responsiveness and biodegradability. The versatility of building blocks provides a library of polymers with miscellaneous physicochemical and mechanical properties. When used alone or together with other materials, PBAEs can be fabricated into different formulations in order to fulfill various requirements in drug delivery (for instance, gene, anticancer drugs, and antimicrobials delivery) and natural complex mimicry (nanochaperones). This progress report discusses the recent developments in design, synthesis, formulations, and applications of PBAEs in biomedical fields and provides a perspective view for the future of the PBAEs.
Graphene Oxide as an Optical Biosensing Platform: A Progress Report
Authors: Morales‐Narváez Eden , Merkoçi Arben
Published: December 2018

A few years ago, crucial graphene oxide (GO) features such as the carbon/oxygen ratio, number of layers, and lateral size were scarcely investigated and, thus, their impact on the overall optical biosensing performance was almost unknown. Nowadays valuable insights about these features are well documented in the literature, whereas others remain controversial. Moreover, most of the biosensing systems based on GO were amenable to operating as colloidal suspensions. Currently, the literature reports conceptually new approaches obviating the need of GO colloidal suspensions, enabling the integration of GO onto a solid phase and leading to their application in new biosensing devices. Furthermore, most GO‐based biosensing devices exploit photoluminescent signals. However, further progress is also achieved in powerful label‐free optical techniques exploiting GO in biosensing, particularly using optical fibers, surface plasmon resonance, and surface enhanced Raman scattering. Herein, a critical overview on these topics is offered, highlighting the key role of the physicochemical properties of GO. New challenges and opportunities in this exciting field are also highlighted.
A Magnetic Gated Nanofluidic Based on the Integration of a Superhydrophilic Nanochannels and a Reconfigurable Ferrofluid
Authors: Wang Dianyu , Zheng Shuang , Liu He , Tang Jiayue , Miao Weining , Wang Huanting , Tian Ye , Yang Hua , Jiang Lei
Published: December 2018

The design of intelligent gating in nanoscale is the subject of intense research motivated by a broad potential impact on science and technology. However, the existing designs require complex modification and are unstable, which restrict their practical applications. Here, a magnetic gated nanofluidic is reported based on the integration of superhydrophilic membranes and reconfigurable ferrofluid, which realizes the gating of the nanochannel by adjusting the steric configuration of the ferrofluid. This system could achieve ultrahigh gating ratio up to 10 000 and excellent stability up to 130 cycles without attenuation. Experiments and theoretical calculations demonstrate that the switch is controlled by the synergy of magnetic force and the interfacial tension. The introduction of ferrofluid and superhydrophilic nanochannels in this work presents an important paradigm for the nanofluidic systems and opens a new and promising avenue to various developments in the fields of materials science, which may be utilized in medical devices, nanoscale synthesis, and environmental analysis.
Luxury of N‐Tosylhydrazones in Transition‐Metal‐Free Transformations
Authors: Arunprasath Dhanarajan , Devi Bala Balasubramanian , Sekar Govindasamy
Published: December 2018

This review is focused on the recent developments in transition‐metal‐free transformations using N‐tosylhydrazones, emerging building blocks in contemporary organic chemistry. Emphasis is given to their synthetic potential, scope, mechanism and limitations. N‐Tosylhydrazones, derivatives of carbonyl compounds, can undergo a wide range of reactions under transition‐metal‐free reaction conditions. The chemical behaviour of tosylhydrazones is unique under different reaction conditions as they can act as diazo precursors, carbene sources, 1,3‐dipoles, ambiphilic reagents, one‐carbon synthons, and an alternative to the parent carbonyl compounds. These diverse reactivities have resulted in the development of novel C−C and C−heteroatom bond‐forming reactions. The potential of N‐tosylhydrazones in transition‐metal‐free reactions is evident from the synthesis of a handful of molecules such as cyclopropanes, di‐ and triarylmethanes, pyrazoles, triazoles, pyridazines, ethers, carbamates, thioacetals, thiadiazoles and many others.

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Structure and water permeability and resistance characteristics of N‐isopropylacrylamide‐based polymer/polyurethane resin films
Authors: Enomoto Masao , Omote Yuichiro , Miyajima Mitsuo , Yun Che Kyung
Published: December 2018

In this study, moisture‐permeable waterproof film materials exhibiting temperature‐sensitive hydrophilicity are investigated. The water resistance properties of copolymer films prepared by combining water‐soluble poly(N‐isopropylacrylamide) (poly‐NIPAM) with hydrophilic polyurethane (PU) are examined. Water‐soluble poly‐NIPAM is synthesized as a diol and then copolymerized with PU. Structural investigations confirm that water‐soluble poly‐NIPAM and hydrophilic PU are combined through PU links. In terms of physical properties, the films are confirmed to be water‐insoluble. Furthermore, based on the relationship between the water‐swelling ratio of the film and the water temperature, the films exhibit temperature‐sensitive hydrophilicity, with the water‐swelling ratio changing across the lower critical solution temperature (LCST). Moreover, the drying rate of the water‐absorbing film is different across the LCST. From these results, it is confirmed that a functional film with temperature‐dependent hydrophilicity is achieved. This property enables the poly‐NIPAM/PU copolymerized films to be used as moisture‐permeable waterproof fabrics for sportswear.
GPR126: A novel candidate gene implicated in autosomal recessive intellectual disability
Authors: Hosseini Masoumeh , Fattahi Zohreh , Abedini Seyedeh Sedigheh , Hu Hao , Ropers Hans‐H. , Kalscheuer Vera M. , Najmabadi Hossein , Kahrizi Kimia
Published: December 2018

Intellectual disability (ID), a genetically and clinically heterogeneous disorder, affects 1%–3% of the general population and is a major health problem, especially in developing countries and in populations with a high frequency of consanguineous marriage. Using whole exome sequencing, a homozygous missense variation (c.3264G>C, p.W1088C) in a plausible disease causing gene, GPR126, was identified in two patients presenting with profound ID, severe speech impairment, microcephaly, seizures during infancy, and spasticity accompanied by cerebellar hypoplasia. The role of GPR126 in radial sorting and myelination in Schwann cells suggests a mechanism of pathogenesis for ID. Involvement of GPR126 in lethal congenital contracture syndrome 9 has been identified previously, but this is the first report of a plausible candidate gene, GPR126, in ID.
A novel autosomal dominant mutation in SOX18 resulting in a fatal case of hypotrichosis–lymphedema–telangiectasia syndrome
Authors: Wangberg Hannah , Wigby Kristen , Jones Marilyn C.
Published: December 2018

Hypotrichosis–lymphedema–telangiectasia syndrome (HLTS) is a rare disorder caused by autosomal recessive and autosomal dominant mutations in SOX18. This gene encodes a transcription factor involved in the regulation and development of the human vasculature, lymphatic, and integumentary systems. Individuals with HLTS develop varying degrees of hypotrichosis, lymphedema, and telangiectasias. Other complications, such as renal failure and aortic dilation, have also been observed. Here, we report a neonate with a novel mutation in SOX18 (c.541C>T; p.Gln181stop) presenting with cardinal features of HLTS in addition to unique findings of severe chylothorax and relentless pulmonary hypertension that culminated in death. The purpose of this report is to summarize what is known about this evolving genetic syndrome and to speculate as to how mutations in SOX18 might produce the phenotype.
A case of YY1‐associated syndromic learning disability or Gabriele‐de Vries syndrome with myasthenia gravis
Authors: Morales‐Rosado Joel A. , Kaiwar Charu , Smith Benn E. , Klee Eric W. , Dhamija Radhika
Published: December 2018

Exome sequencing is being used increasingly to evaluate patients with intellectual disability. YY1 is a ubiquitously distributed transcription factor belonging to the GLIKruppel class of zinc finger proteins recently recognized as the causative gene in 23 patients for the Gabriele‐de Vries syndrome. We report a new case with similar features and a novel variant in YY1, in a region of the gene, which has not previously been reported. A 25 year old female was referred to clinical genetics with a diagnosis of autoimmune myasthenia gravis, facial dysmorphism and learning disability. Chromosomal microarray and gene panel test for congenital myasthenic syndrome was negative. Whole exome sequencing (WES) revealed a presumed pathogenic de novo novel, heterozygous, truncating variant in the YY1 gene, c.860_864delTTAAAA, p.Ile287Argfs*3. The Ile287 residue is conserved across species and is situated in the transcription repressor domain of the protein. This variant is novel and lies in a domain of the protein where no previously reported variants occur. The phenotypic features of our case closely match those of the reported patients. Autoimmune myasthenia gravis has not been reported in these patients and may constitute an expansion of this phenotypic spectrum or perhaps more likely a second unrelated diagnosis.
In Situ Electrochemical Conversion of an Ultrathin Tannin Nickel Iron Complex Film as an Efficient Oxygen Evolution Reaction Electrocatalyst
Authors: Shi Yanmei , Yu Yu , Liang Yu , Du Yonghua , Zhang Bin
Published: December 2018

The oxygen evolution reaction (OER) is an important half reaction in many energy conversion and storage techniques. However, the development of a low‐cost easy‐prepared OER electrocatalyst with high mass activity and rapid kinetics is still challenging. Herein, we report the facile deposition of tannin‐NiFe (TANF) complex film on carbon fiber paper (CP) as a highly efficient OER electrocatalyst. TANF gives rapid OER reaction kinetics with a very small Tafel slope of 28 mV dec−1. The mass activity of TANF reaches 9.17×103 Ag−1 at an overpotential of 300 mV, which is nearly 200‐times larger than that of NiFe double layered hydroxide. Furthermore, tannic acid in TANF can be electrochemically extracted under anodic potential, leaving the inorganic composite NixFe1−xOyHz as the OER‐active species. This work may provide a guide to probing the electrochemical transformation and investigating the reactive species of other metal–organic complexes as heterogeneous electrocatalysts.
Unbiased Rotational Motions of an Ellipsoidal Guest in a Tight Yet Pliable Host
Authors: Sun Zhe , Mio Tatsuru , Okada Tomohiko , Matsuno Taisuke , Sato Sota , Kono Hirohiko , Isobe Hiroyuki
Published: December 2018

In the design of machinery such as steel bearings, a fundamental understanding of material characteristics provides an indispensable basis for the design. Although hydrocarbon cycloarylenes have started to be used for providing unique supramolecular bearings with anomalous dynamic behaviors, their fundamental understanding is immature. A unique property of the cycloarylene host is now reported: the cyclic host is so pliable that it tracks the orientational changes of the ellipsoidal guest, that is, C70 fullerene. Unique structures of the complex were revealed by spectroscopic and crystallographic analyses, and additional theoretical investigations deepened our understanding by revealing the structural changes associated with unbiased rotational motions.
Facile Formation of β‐thioGlcNAc Linkages to Thiol‐Containing Sugars, Peptides, and Proteins using a Mutant GH20 Hexosaminidase
Authors: Tegl Gregor , Hanson John , Chen Hong‐Ming , Kwan David H , Santana Andrés G. , Withers Stephen G.
Published: December 2018

Thioglycosides are hydrolase‐resistant mimics of O‐linked glycosides that can serve as valuable probes for studying the role of glycosides in biological processes. The development of an efficient, enzyme‐mediated synthesis of thioglycosides, including S‐GlcNAcylated proteins, is reported, using a thioglycoligase derived from a GH20 hexosaminidase from Streptomyces plicatus in which the catalytic acid/base glutamate has been mutated to an alanine (SpHex E314A). This robust, easily‐prepared, engineered enzyme uses GlcNAc and GalNAc donors and couples them to a remarkably diverse set of thiol acceptors. Thioglycoligation using 3‐, 4‐, and 6‐thiosugar acceptors from a variety of sugar families produces S‐linked disaccharides in nearly quantitative yields. The set of possible thiol acceptors also includes cysteine‐containing peptides and proteins, rendering this mutant enzyme a promising catalyst for the production of thio analogues of biologically important GlcNAcylated peptides and proteins.
A Three‐Dimensional Dynamic Supramolecular “Sticky Fingers” Organic Framework
Authors: Fernandez‐Bartolome Estefania , Santos José , Gamonal Arturo , Khodabakhshi Saeed , McCormick Laura J. , Teat Simon J. , Sañudo E. Carolina , Costa José Sánchez , Martín Nazario
Published: December 2018

Engineering high‐recognition host–guest materials is a burgeoning area in basic and applied research. The challenge of exploring novel porous materials with advanced functionalities prompted us to develop dynamic crystalline structures promoted by soft interactions. The first example of a pure molecular dynamic crystalline framework is demonstrated, which is held together by means of weak “sticky fingers” van der Waals interactions. The presented organic‐fullerene‐based material exhibits a non‐porous dynamic crystalline structure capable of undergoing single‐crystal‐to‐single‐crystal reactions. Exposure to hydrazine vapors induces structural and chemical changes that manifest as toposelective hydrogenation of alternating rings on the surface of the [60]fullerene. Control experiments confirm that the same reaction does not occur when performed in solution. Easy‐to‐detect changes in the macroscopic properties of the sample suggest utility as molecular sensors or energy‐storage materials.
Ambulatory Care Sensitive Admissions in Individuals With Autism Spectrum Disorder, Intellectual Disability, and Population Controls
Authors: Hand Brittany N. , Boan Andrea D. , Bradley Catherine C. , Charles Jane M. , Carpenter Laura Arnstein
Published: December 2018

Ambulatory care sensitive (ACS) admissions are those for which effective primary care can prevent the need for emergency department (ED) visits and inpatient hospitalizations, and are an indicator of primary care access. Individuals with autism spectrum disorder (ASD) and/or intellectual disability (ID) may be at higher risk for ACS admissions than individuals in the general population due to difficulty accessing primary care. The objective of this study was to compare the incidence of ACS admissions among four cohorts of individuals aged 2–24 years: ASD without co‐occurring ID (ASD‐only), ASD with co‐occurring ID (ASD + ID), ID without ASD (ID‐only), and population controls (PC). Data from ED visits and inpatient hospitalizations occurring between January 1, 2000 and December 31, 2015 were examined to identify ACS admissions. Generalized linear models were used to examine differences between cohorts on the number of ACS ED visits and inpatient hospitalizations. Results revealed the ASD + ID and ID‐only cohorts had significantly higher rates of ACS inpatient hospitalizations than the PC cohort. Additionally, the ID‐only cohort had higher rates of ACS ED visits than the PC cohort. The ASD‐only and PC cohorts did not differ on incidence of ACS admissions. These findings suggest that presence of an ID with or without co‐occurring ASD increased the risk for ACS inpatient hospitalizations, and presence of ID‐only increased the risk for ACS ED visits. Future work should examine trajectories of ACS admissions over time and consider inclusion of additional characteristics that may elucidate reasons for differences in ACS admissions among these groups. Autism Res 2019, 12: 295–302 © 2018 International Society for Autism Research, Wiley Periodicals, Inc.

Lay Summary

Preventable hospitalizations are a common indicator of problems with access to quality primary healthcare. Findings of this study suggest that individuals with intellectual disability, with or without autism spectrum disorder, have higher rates of preventable hospitalizations than the general population. Further research is needed to understand how to improve access to primary care and reduce preventable hospitalizations for this vulnerable population.
An assessment of the impact of green tea extract on palbociclib pharmacokinetics using a validated UHPLC–QTOF–MS method
Authors: Paul David , Surendran Shruti , Chandrakala Patheparapu , Satheeshkumar Nanjappan
Published: December 2018

Green tea extracts (GTE) has been reported to be a kinase inhibitor and modulator for various drug metabolizing enzymes. It may give synergetic antioncogenic effect, but with a possibility of pharmacokinetic interactions with various co‐administered anticancer agents like palbociclib (PAL), a selective inhibitor of CDK‐4/6 primarily metabolized by CYP3A enzyme. To explore the impact of GTE on PAL pharmacokinetics in Sprague–Dawley rats, a rapid and sensitive UHPLC–QTOF–MS method was established. Chromatographic separation was carried out on an Acquity UPLC BEH C18 (100 × 2.1 mm, 1.7 μm) column using a gradient mobile phase system consisting of 0.1% formic acid and acetonitrile. Sample preparation was based on a simple protein precipitation method. Estimation of target ions [M + H]+ at m/z 448.2455 for PAL and m/z 441.2044 for ibrutinib (IS) was performed in selective ion mode ESI–HRMS. Good sensitivity (1.0 ng/mL) and linearity over a wide concentration range of 1–2000 ng/mL was exhibited by the method. The results indicated that the administration of GTE resulted in decreased oral bioavailability of PAL in both short‐ and long‐term conditions. However, when both conditions were compared, the variation was less for the peak concentration and area under the concentration–time curve level of PAL.
Synthesis and Biological Evaluation of Sigma‐1 (σ1) Receptor Ligands Based on Phenyl‐1,2,4‐oxadiazole Derivatives
Authors: Cao Xudong , Yao Zhongyuan , Dou Fei , Zhang Yifang , Qiu Yinli , Zhao Song , Xu Xiangqing , Liu Xin , Liu Bi‐Feng , Chen Yin , Zhang Guisen
Published: December 2018

In this study, a series of phenyl‐1,2,4‐oxadiazole derivatives were synthesized and evaluated for anti‐allodynic activity. Structure–activity relationship studies identified 1‐{4‐[3‐(2,4‐dichlorophenyl)‐1,2,4‐oxadiazol‐5‐yl]butyl}piperidine (39) with excellent affinity for the σ1 receptor and selectivity for the σ2 receptor, with poor activity to other central nervous system neurotransmitter receptors and transporters associated with pain. Compound 39 exhibited dose‐dependent efficacy in suppressing the formalin‐induced flinching and attenuating mechanical allodynia in chronic constriction injury‐induced neuropathic rats. These results suggest that compound 39 exerts potent antihyperalgesic activity and could be considered as a promising candidate for treating neuropathic pain.
Several Polyphosphate Kinase 2 Enzymes Catalyse the Production of Adenosine 5′‐Polyphosphates
Authors: Mordhorst Silja , Singh Jyoti , Mohr Michael K. F. , Hinkelmann Rahel , Keppler Michael , Jessen Henning J. , Andexer Jennifer N.
Published: December 2018

Polyphosphate kinases (PPKs) are involved in many metabolic processes; enzymes of the second family (PPK2) are responsible for nucleotide synthesis fuelled by the consumption of inorganic polyphosphate. They catalyse the phosphorylation of nucleotides with various numbers of phosphate residues, such as monophosphates or diphosphates. Hence, these enzymes are promising candidates for cofactor regeneration systems. Besides adenosine 5′‐triphosphate, PPK2s also catalyse the synthesis of highly phosphorylated nucleotides in vitro, as shown here for adenosine 5′‐tetraphosphate and adenosine 5′‐pentaphosphate. These unusually phosphorylated adenosine 5′‐polyphosphates add up to 50 % of the whole adenosine nucleotides in the assay. The two new products were chemically synthesised to serve as standards and compared with the two enzymatically produced compounds by high‐performance ion chromatography and 31P NMR analysis. This study shows that PPK2s are highly suitable for biocatalytic synthesis of different phosphorylated nucleotides.
Low‐dose systemic thrombolytic therapy for treatment of submassive pulmonary embolism: Clinical efficacy but attendant hemorrhagic risks
Authors: Rothschild Daniel P. , Goldstein James A. , Bowers Terry R.
Published: December 2018

Objectives

The purpose of the present study is to evaluate the safety and efficacy of “low‐dose” systemic thrombolytic therapy (TT) for treatment of patients with intermediate‐high risk submassive pulmonary embolism (PE).

Background

TT is increasingly utilized in acute submassive PE. Strategies for TT include catheter‐directed administration as well as traditional IV systemic therapy. Regardless of the route, most studies document the attendant significant bleeding complication rates expected from induction of a systemic lytic state. To mitigate bleeding, “low‐dose” systemic TT (Alteplase 50 mg) has been advocated, based on recent studies which demonstrated clinical efficacy with elimination of any significant bleeding complications.

Methods

Over a 24‐month period, our institutional PE Response Team treated 45 acute submassive PE patients with “Low Dose” IV Alteplase 50 mg. Clinical outcomes and bleeding complications were assessed.

Results

Overall clinical outcome was excellent, with 97.8% of patients surviving to discharge and a 30‐day, all‐cause mortality of 4.4%. Despite no patients having a HAS‐BLED score > 2 (average score = 0.8 +/−), ISTH major and GUSTO moderate bleeding was observed in 11% (n = 5) of cases.

Conclusions

The present observations document that low‐dose systemic TT is associated with excellent clinical outcome for intermediate‐high risk submassive PE, but with attendant risk for bleeding. These findings are consistent with the concept that induction of a therapeutic lytic state carries inextricable bleeding risk.
A Self‐Assembled Unit Comprising 12 Squaraine Dyes Built Up from Two Star‐Shaped Hexasquarainyl‐Benzene Molecules
Authors: Schreck Maximilian H. , Röhr Merle I. S. , Clark Timothy , Stepanenko Vladimir , Würthner Frank , Lambert Christoph
Published: December 2018

We describe the aggregate formation and optical properties of a star‐shaped hexaarylbenzene with six squaraine chromophores (=hexasquarainyl benzene). Comprehensive concentration‐dependence studies in acetone/CHCl3 mixtures reveal a strong propensity to form discrete dimeric aggregates with a high binding constant in excess of 106 m−1. In this context, a large hypsochromic shift of almost 2700 cm−1 was found in the absorption spectrum, indicating H‐type exciton coupling. The aggregate band is characterised by a very small band width of only 560 cm−1, probably caused by exchange narrowing. Both experimental and computational methods were used to elucidate the supramolecular aggregate structure, which is assumed to consist of two stacked hexasquarainyl benzene monomers.
Silver Nanorings: New Generation of Transparent Conductive Films
Authors: Azani Mohammad‐Reza , Hassanpour Azin
Published: December 2018

Invited for the cover of this issue is the group of Mohammad‐Reza Azani and Azin Hassanpour from Intercomet S.L., Madrid, Spain. The image depicts nanowires converting to nanorings with better opto‐electrical properties. Read the full text of the article at 10.1002/chem.201804788.