bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deleterious Germline BLM Mutations and the Risk for Early-onset Colorectal Cancer
Authors: de Voer Richarda M. , Hahn Marc-Manuel , Mensenkamp Arjen R. , Hoischen Alexander , Gilissen Christian , Henkes Arjen , Spruijt Liesbeth , van Zelst-Stams Wendy A. , Marleen Kets C. , Verwiel Eugene T. , Nagtegaal Iris D. , Schackert Hans K. , van Kessel Ad Geurts , Hoogerbrugge Nicoline , Ligtenberg Marjolijn J.L. , Kuiper Roland P. 
Published: September 2015

AbstractBloom syndrome is an autosomal recessive disorder characterized by chromosomal instability and increased cancer risk, caused by biallelic mutations in the RECQL-helicase gene BLM. Previous studies have led to conflicting conclusions as to whether carriers of heterozygous BLM mutations have an increased risk to develop colorectal cancer (CRC). We recently identified two carriers of a pathogenic BLM mutation in a cohort of 55 early-onset CRC patients (≤45 years of age), suggesting an overrepresentation compared to the normal population. Here, we performed targeted sequencing using molecular inversion probes to screen an additional cohort of 185 CRC patients (≤50 years of age) and 532 population-matched controls for deleterious BLM mutations. In total, we identified three additional CRC patients (1.6%) and one control individual (0.2%) that carried a known pathogenic BLM mutation, suggesting that these mutations are enriched in early-onset CRC patients (P = 0.05516). A comparison with local and publically available databases from individuals without suspicion for hereditary cancer confirmed this enrichment (P = 0.003534). Analysis of family members of the five BLM mutation carriers with CRC suggests an incomplete penetrance for CRC development. Therefore, these data indicate that carriers of deleterious BLM mutations are at increased risk to develop CRC, albeit with a moderate-to-low penetrance.
Composite materials with enhanced dimensionless Young’s modulus and desired Poisson’s ratio
Authors: Zhu H. X. , Fan T. X. , Zhang D. 
Published: September 2015

AbstractWe have designed a new type of composite materials which not only has a Young’s modulus much larger than the Voigt limit, but also is always nearly isotropic. Moreover, its Poisson’s ratio can be designed at a desired value, e.g. positive, or negative, or zero. We have also demonstrated that structural hierarchy can help to enhance the stiffness of this type of composite materials. The results obtained in this paper provide a very useful insight into the development of new functional materials and structures.
Current Insights into the Pathogenesis of Graves’
Ophthalmopathy
Authors: Bahn R. S. 
Published: September 2015

AbstractEnvironmental, genetic, and immune factors are at play in the development of the
variable clinical manifestations of Graves’ ophthalmopathy (GO). Among the
environmental contributions, smoking is the risk factor most consistently linked
to the development or worsening of the disease. The close temporal relationship
between the diagnoses of Graves’ hyperthyroidism and GO have long suggested that
these 2 autoimmune conditions may share pathophysiologic features. The finding
that the thyrotropin receptor (TSHR) is expressed in orbital fibroblasts, the
target cells in GO, supported the notion of a common autoantigen. Both cellular
and humeral immunity directed against TSHR expressed on orbital fibroblasts
likely initiate the disease process. Activation of helper T cells recognizing
TSHR peptides and ligation of TSHR by TRAb lead to the secretion of inflammatory
cytokines and chemokines, and enhanced hyaluronic acid (HA) production and
adipogenesis. The resulting connective tissue remodeling results in varying
degrees extraocular muscle enlargement and orbital fat expansion. A subset of
orbital fibroblasts express CD34, are bone-marrow derived, and circulate as
fibrocytes that infiltrate connective tissues at sites of injury or
inflammation. As these express high levels of TSHR and are capable of producing
copious cytokines and chemokines, they may represent an orbital fibroblast
population that plays a central role in GO development. In addition to TSHR,
orbital fibroblasts from patients with GO express high levels of IGF-1R. Recent
studies suggest that these receptors engage in cross-talk induced by TSHR
ligation to synergistically enhance TSHR signaling, HA production, and the
secretion of inflammatory mediators.
Population Dynamics of the Cui-ui of Pyramid Lake, Nevada: a Potamodromous Catostomid Subject to Failed Reproduction
Authors: Scoppettone G. Gary , Rissler Peter H. , Fabes Mark C. , Shea Sean P. 
Published: September 2015

Abstract
Fishes of the Truckee River basin (California and Nevada) evolved in an aquatic system that has been episodically diminished by extended drought. For potamodromous species, such as the endangered Cui-ui endemic to Pyramid Lake, Nevada, prehistoric episodic severe drought presumably led to periods of failed reproduction due to restricted access to spawning habitat. The response of the Cui-ui population to more recent failed reproduction caused by anthropogenic activity was studied to learn how to manage this species through periods of spawning disruption. Adult Cui-ui survival averaged 91% and 89% for females and males, respectively, in drought years when spawning migrations were either precluded or few fish migrated because of no or low stream flow. In each of 2 years when stream access was precluded, the adult survival was nearly 100% suggesting that Cui-ui survival is extended in the absence of a spawning migration. Survival averaged 62% and 60% for females and males, respectively, in years of spawning migrations. Strong predominant year-classes developed in the year immediately following a period of failed reproduction, indicating the species' capacity for population rebound. Year-class predominance persisted for 6–10 years and through years of low survival associated with migration years, and this predominance is probably due, in part, to a diverse age at maturity. Contemporary water diversions from the Truckee River provided the opportunity to study the response of the Cui-ui population to years of failed reproduction. A projected drier Truckee River basin associated with global climate change will test the Cui-ui's adaptive capacity to endure periods of reproductive failure. This study is aimed at assisting Cui-ui managers in conserving the species in this highly regulated and changing system. The study also adds insight into the prehistoric population dynamics of a potamodromous species in the arid western United States subject to wide fluctuations in annual precipitation and water availability.
Received January 27, 2015; accepted May 22, 2015
SeDuS: segmental duplication simulator
Authors: Hartasánchez Diego A. , Brasó-Vives Marina , Fuentes-Díaz Juanma , Vallès-Codina Oriol , Navarro Arcadi 
Published: September 2015

Abstract
Summary: SeDuS is the first flexible and user-friendly forward-in-time simulator of patterns of molecular evolution within segmental duplications undergoing interlocus gene conversion and crossover. SeDuS introduces known features of interlocus gene conversion such as biased directionality and dependence on local sequence identity. Additionally, it includes aspects such as different selective pressures acting upon copy number and flexible crossover distributions. A graphical user interface allows fast fine-tuning of relevant parameters and straightforward real-time analysis of the evolution of duplicates.
Availability and implementation: SeDuS is implemented in C++ and can be run via command line or through a graphical user interface developed using Qt C++. Source code and binary executables for Linux, OS X and Windows are freely available at www.biologiaevolutiva.org/sedus/. A tutorial with a detailed description of implementation, parameters and output files is available online.
Contact: arcadi.navarro@upf.edu
The language of epistemic specifications (refined) including a prototype solver
Authors: Kahl Patrick , Watson Richard , Balai Evgenii , Gelfond Michael , Zhang Yuanlin 
Published: September 2015

Abstract
In this article, we present a new version of the language of Epistemic Specifications. The goal is to simplify and improve the intuitive and formal semantics of the language. We describe an algorithm for computing solutions of programs written in this new version of the language. The new semantics is illustrated by a number of examples, including an Epistemic Specifications-based framework for conformant planning. In addition, we introduce the notion of an epistemic logic program with sorts . This extends recent efforts to define a logic programming language that includes the means for explicitly specifying the domains of predicate parameters. An algorithm and its implementation as a solver for epistemic logic programs with sorts is also discussed.
Hate Crimes
Authors: Schweppe Jennifer , Walters Mark Austin 
Published: September 2015

AbstractThis article analyzes the current legislative approach to combating hate crime. Part I starts with an overview of the key theoretical arguments for and against the use of punishment enhancements for hate crime offenders. Both retribution and consequentialist theories of punishment are examined in detail in order to evaluate whether the increased punishment of hate-motivated offenders can be justified. Part II then outlines the various models of legislation that have been used to legislate for hate crime globally. Hate crime provisions in several jurisdictions (United States, Canada, England and Wales) are examined in order to explore the practical differences between the various models of legislation that have emerged. The final part of the article outlines the practical problems that are frequently faced by law enforcers and prosecutors of hate crime offenses, including the evidential difficulties posed by the inclusion of hate motivation within the law.
High-Visibility Policing
Authors: Sandhu Ajay , Haggerty Kevin D. 
Published: September 2015

Abstract
The public now expects that police work will be video recorded. This challenges the foundational axiom that police work is a “low-visibility” occupation. Technological and social developments are rapidly making aspects of police work increasingly visible. A consensus seems to be emerging that this new high-visibility status undermines public trust and challenges police legitimacy. This article analyzes this situation and questions the extent to which videos of the police are producing uniformly negative outcomes for them. It emphasizes how the police are involved in an ongoing struggle for legitimacy, played out on a public stage and involving different images, audiences, and interpretations of recorded police behavior, paying attention to the complex and countervailing dynamics of police visibility, empowerment, and legitimacy. It highlights some of these dimensions by drawing on research on policing in Canada to accentuate the ways individual line officers relate to their heightened camera visibility.
Tibetan Buddhist Self-Immolation
Authors: Carrico Kevin 
Published: September 2015

Abstract
Since 2009, 141 Tibetans have engaged in self-immolation, setting their bodies alight, in protest against China’s rule of their homeland. This article asks why. How has this previously unknown form of protest become the primary symbol of political opposition in Tibet today? Noting the lack of a tradition of self-immolation in Tibetan Buddhist culture, this article finds the origins of this seemingly incomprehensible act within the current sociopolitical context, wherein this fundamentally new phenomenon has taken on significant symbolic meaning in just a few years. This article further analyzes political, somatic, and religious meanings employed in Tibetan communities in interpreting this act, demonstrating how communities make sense of this phenomenon’s intertwined power and horror. Finally, beyond the Tibetan community, this article reviews various parties’ responses to these acts of sacrifice to begin envisioning new directions on the Tibetan plateau: a challenge demanded by the act of self-immolation.
Brain Metastases at Presentation in Patients With Non–Small Cell Lung Cancer
Authors: Waqar Saiama N. , Waqar Sadaf H. , Trinkaus Kathryn , Gadea Carlos A. , Robinson Cliff G. , Bradley Jeffrey , Watson Mark A. , Puri Varun , Govindan Ramaswamy , Morgensztern Daniel 
Published: September 2015

Objective:
We used brain radiotherapy as a surrogate for the presence of brain metastases in patients with non–small cell lung cancer (NSCLC) to determine the prevalence of brain metastases using the Surveillance Epidemiology and End Results database.

Methods:
Patients with NSCLC diagnosed between 1988 and 1997 were subdivided according to brain radiotherapy status at presentation into: “none” or “radiation therapy indicated.” We calculated the frequency of brain radiotherapy use in all patients. Odds ratios (ORs) for the indication of brain radiotherapy were calculated for individual prespecified covariates of interest. All statistical tests were 2-sided and P<0.05 were considered significant.

Results:
At presentation, brain radiotherapy was indicated in 10,963 (8.3%) of the 131,456 patients diagnosed with NSCLC between 1988 and 1997. On multivariable analysis the following were significantly associated with brain radiotherapy use: age (OR, 0.653 per 10 y increase in age; 95% confidence interval [CI]: 0.642, 0.665); female sex (OR, 1.05; 95% CI: 1.01, 1.10]); adenocarcinoma histology (HR, 1.67; 95% CI: 1.58, 1.76) or large cell or other histology (OR, 1.67; 95% CI: 1.57, 1.77); tumor size>3 cm (3.1 to 5 cm OR, 1.22; 95% CI: 1.14, 1.30 and >5 cm OR, 1.25; 95% CI: 1.17, 1.33); tumor grade >II (grade III OR, 1.82; 95% CI: 1.69, 1.95 and grade IV OR, 1.91; 95% CI: 1.73, 2.11); and nodal involvement N1 (OR, 1.33; 95% CI: 1.20, 1.47), N2 (OR, 2.24; 95% CI: 2.10, 2.40), and N3 (OR, 2.39; 95% CI: 2.19, 2.60).

Conclusions:
Brain radiotherapy is indicated in over 8% of patients with NSCLC at presentation. We demonstrated that the risk of brain metastasis at presentation may be stratified with the use of 6 clinical factors.
Understanding inequality in public school admission in urban China
Authors: Liu Jing 
Published: September 2015

Purpose
– The purpose of this paper is to apply analysis of public discourses on Ze Xiao to explore and interpret the power relationships shaping inequality in admission to public junior high schools in urban China.

Design/methodology/approach
– This study first introduces the rise of Ze Xiao as an educational phenomenon in China. It then elucidates power relationships in public school admission by analyzing continuities and changes in stakeholders’ interaction in public school admission. It concludes by discussing educational reform for equal public school admission in urban China. Data were collected from written and spoken texts about public school admission, including newspaper articles from the 1980s to the 2000s, policy documents and interviews with relevant stakeholders.

Findings
– Findings demonstrate that multi-layered power relationships caused diverse inequalities in admission to public secondary education in urban China. These are represented by political and institutional privileges and an imbalance in education development during the social transition from a profit-driven approach in the 1990s to a balance-centered one after 2000. Arguably, there is a necessity to further promote a systematic reform to terminate the privileges and imbalance for an equal and balanced public secondary education in urban China post-2015.

Originality/value
– This study attempts to make a contribution toward reconstructing the meaning of inequality in admission to public junior high schools in urban areas by revealing the power relationships among stakeholders constituted through their interactions in public education during the different stages of socio-economic development in urban China.
Neoliberal governance in Indonesian universities: the impact upon academic identity
Authors: Gaus Nurdiana , Hall David 
Published: September 2015

Purpose– The purpose of this paper is to understand the under life of Indonesian academics during ongoing implementation of government-driven policy enacted in higher education instititutions in Indonesia. The attention was specifically focused on the new programme of accountability and quality assurance moderated by the implementation of online assessment system to monitor and evaluate the perfromance of lecturers directly and how this system impacted upon the meaning of academic identity perceived by them.Design/methodology/approach– This study was drawn from a qualitative research of case study approach. Semi-structured interviews were utilised to collect data and conducted with 30 academics from three state universities.Findings– This study revealed that academics were grappling to balance their schism between keeping their existing identity tenable and excercising new prescribed roles from external environment. However, academics were still able to practice their preceived identity through their principled personal project that legitimate them to become academics and pursue their success rather than use instrumental means.Practical implications– The results of this study will be expected to contribute to a better understanding on the dynamics of academics’ world as it is encountered against government-driven policy, and provide indications for policy makers to take into account this issue in the formulation and enactment of their policy.Originality/value– A new aspect of identity in academic profession was found, that is to say religion.
Dynamic capabilities, human resources and operating routines
Authors: Barrales-Molina Vanesa , Montes Francisco Javier Llorens , Gutierrez-Gutierrez Leopoldo J 
Published: September 2015

Purpose– The purpose of this paper is to explain the outcomes and role of dynamic capabilities (DCs). To explain the outcomes, the authors study the relationship between new product development (NPD) (an example of DCs) and metaflexibility. To explain the role of DCs, the authors study how human resources and operating routines moderate the role of DCs in achieving adaptation in the firm.Design/methodology/approach– Using data from 200 managers of Spanish firms, the authors apply regression analysis to test the moderating role of human resources and operating routines in the relationship between NPD and metaflexibility.Findings– The results demonstrate that highly qualified and committed workers enhance the effectiveness of NPD, while high frequency in repetition of operating routines significantly damages such effectiveness.Research limitations/implications– This study is limited to analysing a unique DC (NPD), but future research could explore contributions on other consolidated DCs (e.g. alliance management capability) and compare results. Also, the database on managerial perceptions rather than objective measures.Practical implications– Managers who must address environmental changes should connect generation of DCs to complementary functional strategies, especially human resources strategy.Originality/value– This paper suggests additional outcomes derived from DCs, such as metaflexibility. It attempts to understand the complex process by which DCs interact to modify operating routines in order to respond to environmental changes.
Exchange rate movements and firm value
Authors: Mozumder Nurul , De Vita Glauco , Larkin Charles , Kyaw Khine S. 
Published: September 2015

Purpose
– The purpose of this paper is to investigate the sensitivity of firm value to exchange rate (ER) movements, and the determinants of such exposure for 100 European blue chip companies over 2001-2012.

Design/methodology/approach
– The authors adopt a disaggregated framework that distinguishes between Eurozone and non-Eurozone firms, and between financial and non-financial firms across the pre-crisis, crisis and post-crisis periods of the recent financial crisis.

Findings
– The authors find no significant difference between Eurozone and non-Eurozone, and financial and non-financial firms. Exposure is found to be higher during the financial crisis, across all sub-samples of firms. In the majority of cases the exposure coefficient is significantly positive, indicating that European firms’ stock returns are positively (negatively) affected by depreciation (appreciation) of ERs (indirect quotation).

Practical implications
– It is recommended that firms’ financial plans budget for higher liquidity levels in order to build up, during “good times”, a natural hedge for the higher exposure likely to be faced during periods characterized by greater financial distress.

Originality/value
– The main novelty lies in the adoption of a disaggregated framework that discriminates between pre-crisis, crisis and post-crisis periods in order to ascertain the extent to which the recent financial crisis affected the relationship in question.
Industry level J-curve in Turkey
Authors: Durmaz Nazif 
Published: September 2015

Purpose
– The purpose of this paper is to examine the J-Curve effect in Turkey at the industry level.

Design/methodology/approach
– In order to find the long-run and short-run effects, 58 industries (by Standard International Trade Classification Rev.3) have been identified by using monthly data that covers the periods from January 1990 to December 2012. Present study employs bounds testing procedure, developed by Pesaran and Shin (1999) and Pesaran et al. (2001).

Findings
– Although results indicate a positive satisfactory effect of real depreciation of lira in 13 industries, the J-Curve effect is detected in only 13 industries.

Originality/value
– The present study is one of the first studies to analyze the J-Curve effect at the industry level on Turkey. In addition to being one of the first studies, it will be an invaluable addition to the J-Curve literature.
Research data management services for a multidisciplinary, collaborative research project
Authors: Curdt Constanze , Hoffmeister Dirk 
Published: September 2015

Purpose– Research data management (RDM) comprises all processes, which ensure that research data are well-organized, documented, stored, backed up, accessible, and reusable. RDM systems form the technical framework. The purpose of this paper is to present the design and implementation of a RDM system for an interdisciplinary, collaborative, long-term research project with focus on Soil-Vegetation-Atmosphere data.Design/methodology/approach– The presented RDM system is based on a three-tier (client-server) architecture. This includes a file-based data storage, a database-based metadata storage, and a self-designed user-friendly web-interface. The system is designed in cooperation with the local computing centre, where it is also hosted. A self-designed interoperable, project-specific metadata schema ensures the accurate documentation of all data.Findings– A RDM system has to be designed and implemented according to requirements of the project participants. General challenges and problems of RDM should be considered. Thus, a close cooperation with the scientists obtains the acceptance and usage of the system.Originality/value– This paper provides evidence that the implementation of a RDM system in the provided and maintained infrastructure of a computing centre offers many advantages. Consequently, the designed system is independent of the project funding. In addition, access and re-use of all involved project data is ensured. A transferability of the presented approach to another interdisciplinary research project was already successful. Furthermore, the designed metadata schema can be expanded according to changing project requirements.