bluuebunny/crossref_metadata_2025_split huggingface dataset:
JAK Inhibition in Aicardi-Goutières Syndrome: a Monocentric Multidisciplinary Real-World Approach Study
Authors: Frémond Marie-Louise , Hully Marie , Fournier Benjamin , Barrois Rémi , Lévy Romain , Aubart Mélodie , Castelle Martin , Chabalier Delphine , Gins Clarisse , Sarda Eugénie , Al Adba Buthaina , Couderc Sophie , D’ Almeida Céline , Berat Claire-Marine , Durrleman Chloé , Espil Caroline , Lambert Laetitia , Méni Cécile , Périvier Maximilien , Pillet Pascal , Polivka Laura , Schiff Manuel , Todosi Calina , Uettwiller Florence , Lepelley Alice , Rice Gillian I. , Seabra Luis , Sanquer Sylvia , Hulin Anne , Pressiat Claire , Goldwirt Lauriane , Bondet Vincent , Duffy Darragh , Moshous Despina , Bader-Meunier Brigitte , Bodemer Christine , Robin-Renaldo Florence , Boddaert Nathalie , Blanche Stéphane , Desguerre Isabelle , Crow Yanick J. , Neven Bénédicte 
Published: May 2023

AbstractThe paradigm type I interferonopathy Aicardi-Goutières syndrome (AGS) is most typically characterized by severe neurological involvement. AGS is considered an immune-mediated disease, poorly responsive to conventional immunosuppression. Premised on a chronic enhancement of type I interferon signaling, JAK1/2 inhibition has been trialed in AGS, with clear improvements in cutaneous and systemic disease manifestations. Contrastingly, treatment efficacy at the level of the neurological system has been less conclusive. Here, we report our real-word approach study of JAK1/2 inhibition in 11 patients with AGS, providing extensive assessments of clinical and radiological status; interferon signaling, including in cerebrospinal fluid (CSF); and drug concentrations in blood and CSF. Over a median follow-up of 17 months, we observed a clear benefit of JAK1/2 inhibition on certain systemic features of AGS, and reproduced results reported using the AGS neurologic severity scale. In contrast, there was no change in other scales assessing neurological status; using the caregiver scale, only patient comfort, but no other domain of everyday-life care, was improved. Serious bacterial infections occurred in 4 out of the 11 patients. Overall, our data lead us to conclude that other approaches to treatment are urgently required for the neurologic features of AGS. We suggest that earlier diagnosis and adequate central nervous system penetration likely remain the major factors determining the efficacy of therapy in preventing irreversible brain damage, implying the importance of early and rapid genetic testing and the consideration of intrathecal drug delivery.
“What is language for us?”: Community-based Anishinaabemowin language planning using TEK-nology
Authors: Meighan Paul J. 
Published: May 2023

AbstractLanguage planning and policy (LPP), as a field of research, emerged to solve the “problem” of multilingualism in newly independent nation-states. LPP’s principal emphasis was the reproduction of one-state, one-language policies. Indigenous languages were systematically erased through top-down, colonial medium-of-instruction policies, such as in Canadian residential schools. To this day, ideologies and policies still privilege dominant classes and languages at the expense of Indigenous and minoritized groups and languages. To prevent further erasure and marginalization, work is required at multiple levels. There is growing consensus that top-down, government-led LPP must occur alongside community-led, bottom-up LPP. One shared and common goal for Indigenous language reclamation and revitalization initiatives across the globe is to promote intergenerational language transmission in the home, the community, and beyond. The affordances of digital and online technologies are also being explored to foster more self-determined virtual communities of practice. Following an Indigenous research paradigm, this paper introduces theTEK-nology(Traditional Ecological Knowledge [TEK] and technology) pilot project in the Canadian context.TEK-nologyis an immersive, community-led, and technology-enabled Indigenous language acquisition approach to support Anishinaabemowin language revitalization and reclamation. TheTEK-nologypilot project is an example of bottom-up, community-based language planning (CBLP) where Indigenous community members are the language-related decision-makers. This paper demonstrates that Indigenous-led, praxis-driven CBLP, usingTEK-nology, can support Anishinaabemowin language revitalization and reclamation and more equitable, self-determined LPP. The CBLPTEK-nologyproject has implications for status and acquisition language planning; culturally responsive LPP methodologies; and federal, provincial, territorial, and family language policy.
Impact of women on corporate boards of directors on product quality
Authors: Korenkiewicz Dorota , Maennig Wolfgang 
Published: May 2023

AbstractWe analyze the impact of women on corporate boards of directors on product quality. We innovate firstly by integrating the broad but fragmented research on the topic, offering a first simultaneously testing of a larger set of variables identified to be significant in earlier studies. Second, we add alternative indicators of female representation in board of directors as a potential determinant of product quality. Third, we use evaluation scores of goods by the nonprofit foundation “Stiftung Warentest” as a quality indicator, thus adding to a regionally diversified evidence. We find a significant positive effect of female board directors on product quality.
Why we have duties of autonomy towards marginal agents
Authors: Hirsch Anna 
Published: May 2023

AbstractPatients are usually granted autonomy rights, including the right to consent to or refuse treatment. These rights are commonly attributed to patients if they fulfil certain conditions. For example, a patient must sufficiently understand the information given to them before making a treatment decision. On the one hand, there is a large group of patients who meet these conditions. On the other hand, there is a group that clearly does not meet these conditions, including comatose patients or patients in the late stages of Alzheimer’s disease. Then there is a group of patients who fall into the range in between. At the lower end of this range are so-called ‘marginal agents,’ which include young children and patients in the middle stages of Alzheimer’s disease. They also do not meet the typical requirements for autonomy, which is why they are usually granted fewer autonomy rights. However, some of them are capable of ‘pre-forms’ of autonomy that express what is important to them. These pre-forms differ from mere desires and reflect the identification/authenticity condition of autonomy. They have something in common with autonomous attitudes, choices, and actions – namely, they express thevalueof autonomy. As I will argue, autonomy is a value worthy of protection and promotion – even in its non-reflexive forms. Against this background, it becomes clearwhywe have autonomy duties, more precisely positive, autonomy-enabling duties, towards marginal agents and why we should give them as much attention as autonomy duties towards competent patients.
Seductive details hamper learning even when they do not disrupt
Authors: Kienitz Anna , Krebs Marie-Christin , Eitel Alexander 
Published: May 2023

AbstractPrevious research often revealed detrimental effects of seductive details on learning with multimedia instruction, but there are mixed findings regarding how to best explain these detrimental effects. We investigated whether the detrimental effects of seductive details are mainly mediated by the cognitive processes of diversion (deeper processing of seductive details rather than pertinent content) or disruption (unsuccessful attempts to integrate seductive details with pertinent content) by assessing the effects of instructional prompts. In an online learning experiment, participants (N = 247) learned either without seductive details (control condition) or with seductive details in one of three conditions: Participants received either a prompt informing them about the irrelevance of seductive details (irrelevance-prompt), a prompt to process seductive details and pertinent content separately (separation-prompt), or no prompt within their task instruction. We assessed recall and transfer of knowledge as dependent variables. Supporting the diversion hypothesis, participants in the no-prompt condition regarded seductive details as more relevant and consequently spent more time processing them compared to participants in the irrelevance-prompt condition, which negatively influenced their recall performance. Against the disruption hypothesis, participants in the no-prompt condition reported lower integration avoidance between seductive details and pertinent content compared to participants in the separation-prompt condition, but this led to better rather than worse transfer performance. Our results thus suggest diversion, and not disruption, to be the main process driving the seductive details effect. Reducing the details’ diverting potential seems a good way to deal with seductive details in instruction.
The Effect of Ta, Mg, and Zn Content on the Properties of Ti-Ta-Mg and Ti-Ta-Zn Alloys Prepared by Mechanical Alloying and Hot Pressing
Authors: Kozłowski Mikołaj , Adamek Grzegorz , Siwak Piotr , Jakubowicz Jaroslaw 
Published: May 2023

AbstractThe main goal of this study was to achieve poreless titanium alloys with nanocrystalline structure. To this end, the influence of Ta, Mg and Zn content on the properties of Ti alloys was investigated. At first, nanocrystalline powders of TixTayMg and TixTayZn, where x = (30 and 40 wt.%) and y = (3 and 5 wt.%), were prepared using the mechanical alloying process at room temperature. Then, hot-pressing at 750 °C under vacuum was applied for 10 s to consolidate powders. The structure, microstructure, chemical composition, mechanical properties, corrosion resistance, wettability and MTT assay have been investigated. Alloys with Mg were allowed to undergo a shorter mechanical alloying process, achieve greater grain refinement after consolidation and improve mechanical properties. In all cases with increasing amounts of additives in titanium, more Ti-β phase was available. Furthermore, with increasing elements content, hot-pressed alloys consisting of Mg tended to have a more hydrophobic surface. According to the MTT test, all new alloys show non-toxic properties. Among all alloys tested in this study, Ti40Ta5Mg had the most interesting properties for biomedical applications, the highest content of Ti-β phase (81.3%), lowest porosity (0.07%), lowest Young modulus (102.1 GPa) and the lowest surface free energy (38.45 mN/m).
Heterologous Expression of Plantaricin 423 and Mundticin ST4SA in Saccharomyces cerevisiae
Authors: Rossouw Michelle , Cripwell Rosemary A. , Vermeulen Ross R. , van Staden Anton D. , van Zyl Willem H. , Dicks Leon M. T. , Viljoen-Bloom Marinda 
Published: May 2023

AbstractAntimicrobial peptides or bacteriocins are excellent candidates for alternative antimicrobials, but high manufacturing costs limit their applications. Recombinant gene expression offers the potential to produce these peptides more cost-effectively at a larger scale. Saccharomyces cerevisiae is a popular host for recombinant protein production, but with limited success reported on antimicrobial peptides. Individual recombinant S. cerevisiae strains were constructed to secrete two class IIa bacteriocins, plantaricin 423 (PlaX) and mundticin ST4SA (MunX). The native and codon-optimised variants of the plaA and munST4SA genes were cloned into episomal expression vectors containing either the S. cerevisiae alpha mating factor (MFα1) or the Trichoderma reesei xylanase 2 (XYNSEC) secretion signal sequences. The recombinant peptides retained their activity and stability, with the MFα1 secretion signal superior to the XYNSEC secretion signal for both bacteriocins. An eight-fold increase in activity against Listeria monocytogenes was observed for MunX after codon optimisation, but not for PlaX-producing strains. After HPLC-purification, the codon-optimised genes yielded 20.9 mg/L of MunX and 18.4 mg/L of PlaX, which displayed minimum inhibitory concentrations (MICs) of 108.52 nM and 1.18 µM, respectively, against L. monocytogenes. The yields represent a marked improvement relative to an Escherichia coli expression system previously reported for PlaX and MunX. The results demonstrated that S. cerevisiae is a promising host for recombinant bacteriocin production that requires a simple purification process, but the efficacy is sensitive to codon usage and secretion signals.
Surface morphology influences of PBF-LB manufactured Ti6Al4V parts on adhesive bond strength—investigation of as-built and surface-treated conditions
Authors: Ertürk Emre , Musil Bruno , Diez Gregor , Felber Christian , Höfer Philipp 
Published: May 2023

AbstractAdditive manufacturing in combination with adhesive bonding enables high lightweight potential in structural design. A decisive factor for high adhesive bond performance are the surface properties. In the present work, the surface morphology of laser powder bed fusion (PBF-LB) processed Ti6Al4V parts was investigated in the as-built condition for the build orientations 0°, 45°, 90°. Furthermore, subsequent surface treatment by grit blasting or laser treatment allowed to modify the surface morphology and examine its effect on the bond strength. The surface characteristics were assessed using scanning electron microscopy, laser confocal microscopy and contact angle measurements. Evaluating the bond strength was done for tensile loading by centrifugal adhesion testing. The lowest bond strength was found for 0° oriented specimens, while the 45° and 90° oriented samples showed similar strengths. Fracture surface analysis of the as-built surfaces showed that the partially melted particles remain attached and cause mechanical interlocking. Furthermore, surface treatment by grit blasting allowed a minor improvement of the bond strength, while with laser treatment a significant increase was possible.
Accuracy requirements for the road friction coefficient estimation of a friction-adaptive automatic emergency steer assist (ESA)
Authors: Ahrenhold Tim , Iatropoulos Jannes , Henze Roman 
Published: May 2023

AbstractThe number of traffic accidents resulting in personal injury and property damage is increasingly being reduced by effective advanced driver assistance systems (ADAS). Nevertheless, many traffic accidents still cannot be prevented today because they are due to wet, snow- and ice-covered roads. For this reason, the Institute of Automotive Engineering (IAE) of the Technical University of Braunschweig is investigating the road friction coefficient sensitivity and adaptation of advanced driver assistance systems (ADAS) currently in series production from 2018 to 2021 as part of the ‘Road Condition Cloud’ research project funded by the German Research Foundation (DFG) to increase driving safety, particularly on wet, snow- and ice-covered roads. In this article, the road friction coefficient sensitivity and adaptation of an automatic emergency steer assist is simulatively investigated. This assist overrides the driver to automatically execute an evasive maneuver. The driving maneuver used is a standardized obstacle-avoidance maneuver that is simulatively repeated on a dry, wet, snow- and ice-covered road. The road friction coefficient sensitivity shows that this test is already failed on a wet road because the simulated vehicle does not pass the second lane without errors. Subsequently, a road friction coefficient adaptation of the emergency steer assist is investigated. This adaptation varies the maximum lateral acceleration of the evasive trajectory depending on an estimated value of the road friction coefficient in order not to exceed the maximum adhesion coefficient of the wheels during the evasive maneuver. Ideally, the estimated value matches the true road friction coefficient so that the second lane is passed without errors even on a wet, snow- and ice-covered road. In contrast, an existing difference determines whether the second lane is reached. Finally, the necessary accuracy requirements of the road friction coefficient estimation are determined in an novel estimation error diagram. A road friction coefficient adaptation increases the driving safety of driver advanced assistance systems (ADAS) that are in series production today and future highly automated driving functions (HAF) and is necessary for automated driving because the driver is not present as a fallback level. The described results were presented before in [1].
Effects of perinatal stress on the metabolites and lipids in plasma of dairy goats
Authors: Huang Yan , Kong Yezi , Li Bowen , Zhao Chenxu , Loor Juan J. , Tan Panpan , Yuan Yang , Zeng Fangyuan , Zhu Xiaoyan , Qi Simeng , Zhao Baoyu , Wang Jianguo 
Published: May 2023

AbstractDairy goats experience metabolic stress during the peripartal period, and their ability to navigate this stage of lactation is related to the occurrence and development of metabolic diseases. Unlike dairy cows, there is a lack of comprehensive analysis of changes in the plasma profiles of peripartal dairy goats, particularly using high-throughput techniques. A subset of 9 clinically-healthy dairy goats were used from a cohort of 96 primiparous Guanzhong dairy goats (BCS, 2.75 ± 0.15). Blood samples were collected at seven time points around parturition (d 21, 14, 7 before parturition, the day of kidding, and d 7, 14, 21 postpartum), were analyzed using untargeted metabolomics and targeted lipidomics. The orthogonal partial least squares discriminant analysis model revealed a total of 31 differential metabolites including p-cresol sulfate, pyruvic acid, cholic acid, and oxoglutaric acid. The pathway enrichment analysis identified phenylalanine metabolism, aminoacyl-tRNA biosynthesis, and citrate cycle as the top three significantly-altered pathways. The Limma package identified a total of 123 differentially expressed lipids. Phosphatidylserine (PS), free fatty acids (FFA), and acylcarnitines (ACs) were significantly increased on the day of kidding, while diacylglycerols (DAG) and triacylglycerols (TAG) decreased. Ceramides (Cer) and lyso-phosphatidylinositols (LPI) were significantly increased during postpartum period, while PS, FFA, and ACs decreased postpartum and gradually returned to antepartum levels. Individual species of FFA and phosphatidylcholines (PC) were segregated based on the differences in the saturation and length of the carbon chain. Overall, this work generated the largest repository of the plasma lipidome and metabolome in dairy goats across the peripartal period, which contributed to our understanding of the multifaceted adaptations of transition dairy goats.
The Creation of European Citizenship: Constitutional Miracle or Myopia?
Authors: BARNARD Catherine , LEINARTE Emilija 
Published: May 2023

AbstractEU citizenship, now so central to the European Union's project, remains a highly contested concept in respect of its meaning, its scope, and its purpose. By considering the large body of legal texts and their travaux préparatoires from the 1972 Paris European Council until the adoption of the Maastricht Treaty in 1992, we have explored what the original drafters had in mind in developing the concept of EU citizenship and, crucially, what they did not. The article argues that the notion of European citizenship was seen as a tool to define European identity, and thus to continue the building of the European Union as a whole. European citizenship was thus viewed through a constitutional prism from the outset. The constitutional approach to the concept of European citizenship fed into the wider constitutionalisation project under the Maastricht Treaty and, later, permeated the case law of the Court of Justice of the European Union. We wish to argue that this focus on the constitutional nature of EU citizenship overlooked the more practical implications of citizenship, such as how to manage immigration flows or the infrastructure changes needed in the host state to accommodate a significant number of arrivals of EU citizens (in particular in respect of housing and social welfare benefits). It took more than 20 years after the introduction of EU citizenship for the Court of Justice to become aware of the practical, as opposed to constitutional, implications of the direction of travel it had pursued. By that time, it was too late for the United Kingdom, one of the countries which had received the largest number of EU citizens; the UK voted by a narrow margin to leave the European Union.
Public Law’s Cerberus: A Three-Headed Approach to Charter Rights-Limiting Administrative Decisions
Authors: Stacey Richard 
Published: May 2023

AbstractThis article offers a theoretical and doctrinal solution to a vexing question in public law: how to determine the justifiability of Charter rights-limiting administrative decisions. The jurisprudence suggests three approaches, or modes of reasoning: minimal impairment analysis, ‘interest balancing’, and ‘values-advancing reasoning’. Like Cerberus, the guard dog of Hades, Canadian public law has become three-headed. While scholars and courts argue about which mode of reasoning is categorically best, the culture of justification compels us to ask instead which provides the most compelling explanation for each rights-limiting decision. Just as cutting off one of Cerberus’s heads would diminish his effectiveness as a guard dog, rejecting either of the modes of reasoning would limit decision makers’ capacity to explain their decisions and undermine a culture of justification. The article makes a theoretical case for retaining all three modes of reasoning and sets out a doctrinal approach to determining when each is applicable.
Adverse childhood experiences and craving: Results from an Italian population in outpatient addiction treatment
Authors: Russo Claudio , Bonfiglio Natale Salvatore , Orlando Eva , Falcone Giuseppe , Iuliano Lucia , Grandinetti Antonietta , Acconcia Carmine , Napoletano Adriana , Conte Giada , Landi Barbara , Truono Giovanni , D’Alto Marco , Penna Maria Pietronilla , De Luna Antonio 
Published: May 2023

Abstract

Background
Despite the growing interest in addiction research, which demonstrates the potential predictive role of adverse childhood experiences (ACEs), little is known about their impact on the psychological symptoms of craving.

Methods
After reviewing the relevant diagnostic criteria for addiction and comorbid mental disorders along with routinely collected clinical and service-use data, 208 outpatients were assessed on the study protocol. Following the recruitment phase, nominal and ordinal data were analyzed using nonparametric methods.

Results
Most of the outpatients reported ACEs (89.1%) and experienced cravings (73.4–95.7%). A positive association between ACEs and either intention and preplanning (r = .14, p < .05) or lack of control (r = .15; p < .05) of the craving behavior was found.

Conclusion
Craving behavior in addiction remains a subject of debate. Although correlation analyses showed significant associations between reported ACEs and measures of craving, they were relatively small.