bluuebunny/crossref_metadata_2025_split huggingface dataset:
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed byAslgene transfer
Authors: Baruteau Julien , Perocheau Dany P. , Hanley Joanna , Rocha-Ferreira Eridan , Karda Rajvinder , Ng Joanne , Suff Nattalie , Rahim Ahad A. , Hughes Michael P. , Banushi Blerida , Prunty Helen , Hristova Mariya , Ridout Deborah A. , Virasami Alex , Heales Simon , Howe Stewen J. , Buckley Suzy M. , Mills Philippa B. , Gissen Paul , Waddington Simon N. 
Published: June 2018

ABSTRACTArgininosuccinate lyase (ASL) belongs to the liver-based urea cycle detoxifying ammonia, and the citrulline-nitric oxide cycle synthesising nitric oxide (NO). ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia and a multi-organ disease with neurocognitive impairment. Current therapeutic guidelines aim to control ammonaemia without considering the systemic NO imbalance. Here, we observed a neuronal disease with oxidative/nitrosative stress in ASL-deficient mouse brains. A single systemic injection of gene therapy mediated by an adeno-associated viral vector serotype 8 (AAV8) in adult or neonatal mice demonstrated the long-term correction of the urea cycle and the citrulline-NO cycle in the brain, respectively. The neuronal disease persisted if ammonaemia only was normalised but was dramatically reduced after correction of both ammonaemia and neuronal ASL activity. This was correlated with behavioural improvement and a decrease of the cortical cell death rate. Thus, the cerebral disease in argininosuccinic aciduria involves neuronal oxidative/nitrosative stress not mediated by hyperammonaemia, which is reversed by AAV gene transfer targeting the brain and the liver, acting on two different metabolic pathwaysviaa single vector delivered systemically. This approach provides new hope for hepatocerebral metabolic diseases.
Regulation of spatial and temporal gene expression in an animal germline
Authors: Diag Asija , Schilling Marcel , Klironomos Filippos , Ayoub Salah , Rajewsky Nikolaus 
Published: June 2018

SUMMARYIn animal germlines, regulation of cell proliferation and differentiation is particularly important but poorly understood. Here, using a cryo-cut approach, we mapped RNA expression along theCaenorhabditis elegansgermline and, using mutants, dissected gene regulatory mechanisms that control spatio-temporal expression. We detected, at near single-cell resolution, > 10,000 mRNAs, > 300 miRNAs and numerous novel miRNAs. Most RNAs were organized in distinct spatial patterns. Germline-specific miRNAs and their targets were co-localized. Moreover, we observed differential 3’ UTR isoform usage for hundreds of mRNAs. In tumorousgld-2 gld-1mutants, gene expression was strongly perturbed. In particular, differential 3’ UTR usage was significantly impaired. We propose that PIE-1, a transcriptional repressor, functions to maintain spatial gene expression. Our data also suggest thatcpsf-4andfipp-1control differential 3’ UTR usage for hundreds of genes. Finally, we constructed a “virtual gonad” enabling “virtualin situhybridizations” and access to all data (https://shiny.mdc-berlin.de/spacegerm/).
Base pair editing of goat embryos: nonsense codon introgression into FGF5 to improve cashmere yield
Authors: Li Guanwei , Zhou Shiwei , Li Chao , Cai Bei , Yu Honghao , Ma Baohua , Huang Yu , Ding Yige , Liu Yao , Ding Qiang , He Chong , Zhou Jiankui , Wang Ying , Zhou Guangxian , Li Yan , Yan Yuan , Hua Jinlian , Petersen Bjoern , Jiang Yu , Sonstegard Tad , Huang Xingxu , Chen Yulin , Wang Xiaolong 
Published: June 2018

AbstractThe ability to alter single bases without DNA double strand breaks provides a potential solution for multiplex editing of livestock genomes for quantitative traits. Here, we report using a single base editing system, Base Editor 3 (BE3), to induce nonsense codons (C-to-T transitions) at four target sites in caprine FGF5. All five progenies produced from microinjected single-cell embryos had alleles with a targeted nonsense mutation and yielded expected phenotypes. The effectiveness of BE3 to make single base changes varied considerably based on sgRNA design. Also, the rate of mosaicism differed between animals, target sites, and tissue type. PCR amplicon and whole genome resequencing analyses for off-target changes caused by BE3 were low at a genome-wide scale. This study provides first evidence of base editing in livestock, thus presenting a potentially better method to introgress complex human disease alleles into large animal models and provide genetic improvement of complex health and production traits in a single generation.
Design and assessment of species-level qPCR primers targeting comammox
Authors: Keene-Beach Natalie , Noguera Daniel R. 
Published: June 2018

AbstractPublished PCR primers targeting the ammonia monooxygenase gene (amoA) were applied to samples from activated sludge systems operated with low dissolved oxygen (DO) to quantify total and clade-levelNitrospirathat perform complete ammonium oxidation (comammox); however, we found these existing primers resulted in significant artifact-associated non-target amplification. This not only overestimated comammoxamoAcopies but also resulted in numerous false positive detections in the environmental samples tested, as confirmed by gel electrophoresis. Therefore, to more accurately quantify known comammox, we designed specific and sensitive primers targeting three candidate species:Candidatus(Ca.) Nitrospira nitrosa,Ca.N. inopinata, andCa.N. nitrificans. The primers were tested withamoAtemplates of these candidate species, and used to quantify comammox at the species level in low DO activated sludge systems. We found that comammox related toCa.N. nitrosa were present and abundant in the majority of samples from low DO bioreactors and were not detected in samples from a high DO system. In addition, the greatest abundance ofCa.N. nitrosa was found in bioreactors operated with a long solids retention time.Ca.N. inopinata andCa.N. nitrificans were only detected sporadically in these samples, indicating a minor role of these comammox in nitrification under low DO conditions.Abstract art
Impression management in annual report narratives: the case of the UK private finance initiative
Authors: C. Edgar Victoria , Beck Matthias , Brennan Niamh M. 
Published: June 2018

Purpose
The UK private finance initiative (PFI) public policy is heavily criticised. PFI contracts are highly profitable leading to incentives for PFI private-sector companies to support PFI public policy. This contested nature of PFIs requires legitimation by PFI private-sector companies, by means of impression management, in terms of the attention to and framing of PFI in PFI private-sector company annual reports. The paper aims to discuss this issue.

Design/methodology/approach
PFI-related annual report narratives of three UK PFI private-sector companies, over seven years and across two periods of significant change in the development of the PFI public policy, are analysed using manual content analysis.

Findings
Results suggest that PFI private-sector companies use impression management to legitimise during periods of uncertainty for PFI public policy, to alleviate concerns, to provide credibility for the policy and to legitimise the private sector’s own involvement in PFI.

Research limitations/implications
While based on a sizeable database, the research is limited to the study of three PFI private-sector companies.

Originality/value
The portrayal of public policy in annual report narratives has not been subject to prior research. The research demonstrates how managers of PFI private-sector companies present PFI narratives in support of public policy direction that, in turn, benefits PFI private-sector companies.
The right word for the right crowd: an attempt to recognize the influence of emotions
Authors: Wuillaume Amélie , Jacquemin Amélie , Janssen Frank 
Published: June 2018

PurposeThe purpose of this paper is to propose a better understanding of how entrepreneurial narrative influences resource acquisition in the fundraising context.Design/methodology/approachThe paper combines the literature on emotion as information theory from psychology with behavioral finance findings to develop a conceptual framework with research proposals highlighting the use of narratives in the crowdfunding process.FindingsThe proposition of the paper advocates that entrepreneurial narrative may influence crowdfunders’ attitude and decision to fund a project. It theorizes how emotions in narratives shape the funders’ attitude toward a project and, in turn, their decision to support it. This potential influence is qualified by taking into account the funders’ primary motivations. These motivations affect the degree to which funders rely on affect or cognition to form their attitude and to which they are influenced by more emotional or cognitive narratives.Originality/valueThis framework is the result of an effort to achieve the recognition of emotions in entrepreneurial funding. The paper creates a bridge between the narrative emotional content and the often neglected emotional arousal of funders (considered as traditional investors) to provide a framework for explaining crowdfunders’ decision making. The paper also offers nuances by taking into account the different audiences’ motivations to fund a project.
Lean Six Sigma and the Australian business excellence framework
Authors: Price Oriana M. , Pepper Matthew , Stewart Matthew 
Published: June 2018

Purpose
The purpose of this paper is to examine a contextualized local government case study of the application of Lean Six Sigma (LSS) in conjunction with the Australian Business Excellence Framework (ABEF) to highlight the importance of a good strategic fit between LSS and organizational objectives before implementation.

Design/methodology/approach
A local government council is used in a case study-based approach. Organizational artefacts and documents were used for data collection in conjunction with interviews from senior executives within the organization.

Findings
Results indicate that when used in conjunction with the ABEF, LSS provides focus on organizational learning practices embedded within the implementation of continuous improvement.

Research limitations/implications
The purpose of this paper is to contribute to discourse regarding the effective application and implementation of LLS in local government.

Practical implications
LSS tools and techniques are known to local government, but are applied in isolation of the overarching LSS framework. This paper emphasizes the importance of comprehensive implementation of these tools, guided by the inclusion of an external contextualized framework (ABEF) in conjunction with the LSS to achieve sustainable continuous improvement.

Originality/value
Business excellence frameworks are widely used in the public sector as a reference/means for improvement. This paper highlights the importance of LSS in operationalizing strategic direction provided by such frameworks and providing the focus on learning practices critical for sustainable improvements.
Relative supervisor education and worker well-being
Authors: Artz Benjamin 
Published: June 2018

PurposeLess educated supervisors create worker status incongruence, a violation of social norms that signals advancement uncertainty and job ambiguity for workers, and leads to negative behavioral and well-being outcomes. The purpose of this paper is to compare education levels of supervisors with their workers and measure the correlation between relative supervisor education and worker job satisfaction.Design/methodology/approachUsing the only wave of the 1979 National Longitudinal Survey of Youth that identifies education levels of both supervisor and worker, a series of ordered probit estimates describe the relationship between supervisor education levels and subordinate worker well-being. Extensive controls, sub-sample estimates and a control for sorting confirm the estimates.FindingsWorker well-being is negatively correlated with having a less educated supervisor and positively correlated with having a more educated supervisor. This result is robust to a number of alternative specifications. In sub-sample estimates, workers highly placed in an organization’s hierarchy do not exhibit reduced well-being with less educated supervisors.Research limitations/implicationsA limitation is the inability to control for worker fixed effects, which may introduce omitted variable bias into the estimates.Originality/valueThe paper is the first to introduce relative supervisor–worker education level as a determinant of worker well-being.
Career stages and occupations impacts on workers motivations
Authors: Duarte Henrique , Lopes Diniz 
Published: June 2018

Purpose
The career concept has become fuzzier due to changing work patterns, the ageing workforce and the environmental changes occurring during workers lifespans. Together this requires a renewed and broader reaching contextualization of this concept. The purpose of this paper is to set out an integrative approach arguing that the integration of career stage models with occupational groups proves more explanative of intrinsic and extrinsic worker motivations.

Design/methodology/approach
Secondary data from 23 European countries were drawn from the European Social Survey 2006. The construct validity and reliability of indicators was analyzed. Hypotheses were tested using discriminant analysis.

Findings
Results showed that neither occupations nor career stages are determinants per se of intrinsic motivations, but are better explained by their mutual integration. Career stages were shown to predict per se extrinsic motivations.

Research limitations/implications
The recourse to the European Social Survey pre-determined scales and the application of age ranges as proxies for careers stages suggested the usage of more specific measures in future studies.

Practical implications
Career management and compensation policies might be better tailored to worker motivations by considering the age ranges (as proxies of career stages) and workers’ occupations.

Originality/value
Findings evidenced the explanatory value of occupations for worker motivations and allowed putting into perspective the contextualization of not only boundaryless and protean career concepts, but also career stage theories. Data support the ecological validity of applying a career stages and occupations framework to a highly diversified and representative sample of European countries.
How does labour market history influence the access to hiring interviews?
Authors: Duguet Emmanuel , Le Gall Rémi , L’Horty Yannick , Petit Pascale 
Published: June 2018

PurposeThe purpose of this paper is to provide evidence of the effect of labour market status on the current probability to be invited to a hiring interview. The authors compare the effect of periods of unemployment, part-time job and short-term contracts (STCs).Design/methodology/approachCorrespondence tests were conducted for accountants and sales assistants. The authors estimate the discrimination components from the response rate of each candidate by the asymptotic least squares method.FindingsThe authors find that men with a part-time profile suffer discrimination in both professions. Other differences of treatment are specific: for accountants, the authors find that the probability of success decreases with the time spent in unemployment, while for sales assistants the probability of success is smaller with a history of STCs.Originality/valueThis study compares the effect of different dimensions of career history (part-time versus full-time, permanent versus short-term, unemployment versus employment) for experienced job candidates. It also proposes an alternative way to exploit the design of a correspondence experiment.
Neighborhood signaling effects, commuting time, and employment
Authors: Carlsson Magnus , Reshid Abdulaziz Abrar , Rooth Dan-Olof 
Published: June 2018

PurposeThe purpose of this paper is to investigate whether there is unequal treatment in hiring depending on whether a job applicant signals living in a bad (deprived) neighborhood or in a good (affluent) neighborhood.Design/methodology/approachThe authors conducted a field experiment where fictitious job applications were sent to employers with an advertised vacancy. Each job application was randomly assigned a residential address in either a bad or a good neighborhood. The measured outcome is the fraction of invitations for a job interview (the callback rate).FindingsThe authors find no evidence of general neighborhood signaling effects. However, job applicants with a foreign background have callback rates that are 42 percent lower if they signal living in a bad neighborhood rather than in a good neighborhood. In addition, the authors find that applicants with commuting times longer than 90 minutes have lower callback rates, and this is unrelated to the neighborhood signaling effect.Originality/valueEmpirical evidence of causal neighborhood effects on labor market outcomes is scant, and causal evidence on the mechanisms involved is even more scant. The paper provides such evidence.
Robust adaptive sliding mode control for a human-driven knee joint orthosis
Authors: Bkekri Rihab , Benamor Anouar , Alouane Mohamed Amine , Fried Georges , Messaoud Hassani 
Published: June 2018

Purpose
Assistive technology products are designed to provide additional accessibility to individuals who have physical or cognitive difficulties, impairments and disabilities. The purpose of this paper is to deal with the control of a knee joint orthosis intended to be used for rehabilitation and assistive purpose; this control aims to reduce the influence of the uncertainties and eliminating the external disturbances in the system.

Design/methodology/approach
This paper deals with the robust adaptive sliding mode controller (ASMC) of human-driven knee joint orthosis system with mismatched uncertainties and external disturbances. The shank-orthosis system has been modeled and its parameters have been identified. This control reduces the effect of parameter uncertainties and external disturbances on the system performance and improves the system robustness as results. The ASMC was designed to offer the possibility to track the state of the reference model. Moreover, the Lyapunov stability theory was used to study the asymptotical stability of the ASMC.

Findings
The advantage of the robust ASMC method is the tracking precision and reducing the required time for eliminating external disturbances and uncertainties. The experimental results show in real-time in terms of stability and present that the advantages of this control approach are the position tracking and robustness.

Originality/value
In this paper, to deal with the parameter uncertainties of the human-driven knee joint orthosis, an ASMC was successfully applied based on sliding mode and Lyapunov stability theory. It has good dynamic response and tracking performance. Besides, the adaptive algorithm is simple, easy to achieve and has good adaptability and robustness against the parameter variations and external disturbances. The design technique is simple and efficient. The development of this control takes into consideration the perturbation, allowing to track a desired trajectory.
Causal relationships of moral hazard and adverse selection of Ghanaian Public-Private-Partnership (PPP) construction projects
Authors: Owusu-Manu De-Graft , Kukah A.S. , Edwards David John , Pärn Erika Anneli , El-Gohary Hatem , Aigbavboa Clinton 
Published: June 2018

PurposeThe purpose of this paper was to assess the causal relationship(s) between moral hazard and adverse selection of public–private partnership (PPP) construction projects. Structural equation modelling (SEM) was used to explore the cause and effect relationship between moral hazard and adverse selection problems in PPP construction projects in Ghana. The study produced a framework to predict, estimate and depict the complex causal relationships (i.e. the directionality) between moral hazard and adverse selection.Design/methodology/approachTo test the proposed framework, a quantitative methodology was used, in which, data were collected using research questionnaires that targeted a sample of 280 PPP stakeholders in Ghana. In total, 210 useable questionnaires were retrieved, representing a response rate of 75 per cent.FindingsThe interrelationships between the eight causes and the nine effects of moral hazard and adverse selection were established using the model. The tested framework showed the degree of association and isolation of the unobserved variables on the indicator factors. Confirmatory factor analysis (CFA) was used to evaluate the fit of items to latent constructs. Because the fit of each model was good and the item loadings were adequate, it was assumed that the indicators of the different variables factors were fitting. Furthermore, a diagnostic fit analysis was conducted using the robust maximum likelihood method to test the statistical significance of the parameter estimates.Practical implicationsThis novel research is one of the few studies investigating the causal relationships between moral hazard and adverse selection of PPP construction projects. The research concluded with future studies that seek to validate the model developed in other countries and/or other industries.Originality/valueThe research findings will serve as a guide for construction stakeholders in the PPP sector on the causes and effects of adverse selection and moral hazard and how to mitigate these.
New trends in intellectual capital reporting
Authors: Manes Rossi Francesca , Nicolò Giuseppe , Tartaglia Polcini Paolo 
Published: June 2018

PurposeThe purpose of this paper is to explore a new way to disclose intellectual capital (IC) in universities through their websites. Going beyond traditional tools used for intellectual capital disclosure (ICD), this study aims at identifying possible determinants of ICD via the web.Design/methodology/approachThis paper analyses the institutional websites of a sample of Italian universities adapting the theoretical framework developed by Lowet al.(2015) to the peculiarities of the Italian university system. Moreover, the relationship between certain explanatory factors identified in previous research and the extent of online ICD represented by two disclosure indexes was tested through an ordinary least squares regression model.FindingsThe analysis reveals the extensive use of ICD via websites, especially regarding human and internal capital, while the disclosure of external capital through this means is still limited. Internationality and online visibility both positively affect the extent of a university’s ICD.Research limitations/implicationsThe paper represents the first study investigating online ICD and its determinants in universities, contributing new knowledge to help answer the how and what of the matter.Practical implicationsThe results can serve as encouragement to university managers to enhance online ICD to meet the information needs of a wider audience.Originality/valueThis is the first study to provide evidence about online ICD in universities and to reveal some of the possible determinants to improve this disclosure.