bluuebunny/crossref_metadata_2025_split huggingface dataset:
Non-Skewed X-inactivation Results in NF-κB Essential Modulator (NEMO) Δ-exon 5-autoinflammatory Syndrome (NEMO-NDAS) in a Female with Incontinentia Pigmenti
Authors: Eigemann Jessica , Janda Ales , Schuetz Catharina , Lee-Kirsch Min Ae , Schulz Ansgar , Hoenig Manfred , Furlan Ingrid , Jacobsen Eva-Maria , Zinngrebe Julia , Peters Sarah , Drewes Cosima , Siebert Reiner , Rump Eva-Maria , Führer Marita , Lorenz Myriam , Pannicke Ulrich , Kölsch Uwe , Debatin Klaus-Michael , von Bernuth Horst , Schwarz Klaus , Felgentreff Kerstin 
Published: September 2024

Abstract
Purpose
Genetic hypomorphic defects in X chromosomal IKBKG coding for the NF-κB essential modulator (NEMO) lead to ectodermal dysplasia and immunodeficiency in males and the skin disorder incontinentia pigmenti (IP) in females, respectively. NF-κB essential modulator (NEMO) Δ-exon 5-autoinflammatory syndrome (NEMO-NDAS) is a systemic autoinflammatory disease caused by alternative splicing and increased proportion of NEMO-Δex5. We investigated a female carrier presenting with IP and NEMO-NDAS due to non-skewed X-inactivation.

Methods
IKBKG transcripts were quantified in peripheral blood mononuclear cells isolated from the patient, her mother, and healthy controls using RT-PCR and nanopore sequencing. Corresponding proteins were analyzed by western blotting and flow cytometry. Besides toll-like receptor (TLR) and tumor necrosis factor (TNF) signaling, the interferon signature, cytokine production and X-inactivation status were investigated.

Results
IP and autoinflammation with recurrent fever, oral ulcers, hepatitis, and neutropenia, but no immunodeficiency was observed in a female patient. Besides moderately reduced NEMO signaling function, type I interferonopathy, and elevated IL-18 and CXCL10 were found. She and her mother both carried the heterozygous variant c.613 C > T p.(Gln205*) in exon 5 of IKBKG previously reported in NEMO-deficient patients. However, X-inactivation was skewed in the mother, but not in the patient. Alternative splicing led to increased ratios of NEMO-Dex5 over full-length protein in peripheral blood cell subsets causing autoinflammation. Clinical symptoms partially resolved under treatment with TNF inhibitors.

Conclusion
Non-skewed X-inactivation can lead to NEMO-NDAS in females with IP carrying hypomorphic IKBKG variants due to alternative splicing and increased proportions of NEMO-∆ex5.
A Longitudinal Investigation of the Prevalence and Incidence of Self-Reported COVID-19 Disease and the Pandemic’s Impact Among Seventh-day Adventist and Non-Adventists Living in the UK
Authors: Janko Robert K. , Haussmann Irmgard , Patel Ashok 
Published: September 2024

Abstract
This research investigated the prevalence and incidence of COVID-19 infection among Seventh-day Adventist Christians in the UK compared to non-Adventists and assessed the pandemic’s impact on their health and dietary intake. Seventh-day Adventists and non-Adventists in the UK completed an online survey, including a Food Frequency Questionnaire, a 24-h dietary recall, and health and lifestyle questions. Participants were followed for 2 years to determine COVID-19 incidence rates. The baseline survey was completed by 170 people, 86 of whom were Adventists. The follow-up at 2 years showed a significantly lower self-reported COVID-19 incidence among Adventists (OR 0.45, 95% CI 0.2, 1.0, p = 0.05). The incidence rate among Adventists was 65.48 per 1000 person-years versus 121.79 per 1000 person-years among non-Adventists. Adventists were less likely to experience long COVID (OR 0.30, 95% CI 0.12, 0.78, p = 0.01). Despite being older, Adventists had a significantly lower COVID-19 incidence rate. The Adventist lifestyle, including healthy eating habits, fasting, a plant-based diet, and abstaining from alcohol and coffee, was prevalent in this sample. More research is needed to explore the association between the Adventist lifestyle and infectious disease.
Metal–organic frameworks as optically stimulated luminescence dosimeter
Authors: Joshi Shailesh , Panda Madhusmita , Annalakshmi O. , Srinivas C. Venkata , Venkatraman B. 
Published: September 2024

AbstractMetal–organic frameworks (MOF) have been vigorously investigated as hybrid materials in recent decades due to their exceptional structural versatility and applications in diverse fields. The development of novel MOFs for radiation detection and luminescence-based dosimetry is the latest topic of interest. In this contribution, we have synthesized and characterized a Uranyl MOF, which shows an optically stimulated luminescence (OSL) phenomenon induced by ionizing radiation. OSL dosimetric studies were carried out on Uranyl MOF, which revealed that MOF has a linear dose–response up to 100 Gy with a 1.1 Gy minimum detection dose. It also shows good reusability, around 30% fading in one day, very low fading beyond one day to 30 days, and decent radiation stability (stable up to 10 kGy). Preliminary dosimetry results are promising and show that Uranyl MOF can be utilized in the field of low-dose radiation processing applications in the future.
Big Data and Ideational Institutionalism. Reconsidering the Possibilities and Limitations of Google Ngram in the Study of Ideas
Authors: Kestler Thomas 
Published: September 2024

AbstractIdeas and ideational change are difficult to grasp. When exactly did the idea of sustainability emerge? How has the meaning of democracy changed over time? Did ideational change precede or follow institutional change? These and many similar questions are highly relevant to the emerging field of ideational institutionalism, but they are difficult to answer conclusively without reliable data. Google Books Ngram Viewer, a tool introduced in 2011, opens up new possibilities in this regard by allowing the analysis of millions of print sources over time. However, after initial euphoria, its use has waned somewhat due to growing doubts about its reliability and the validity of the results it produces. Some of these problems have been addressed, but the debate about the potential and limitations of Google Books Ngram Viewer is ongoing. This research note will contribute to this debate by proposing a systematization for the use of search terms based on the concept of belief systems and by presenting the results of a reliability test for the German language corpus.
Does CSR facilitate access to trade credit? The role of family ownership
Authors: Rivera-Franco Pilar , Requejo Ignacio , Suárez-González Isabel 
Published: September 2024

AbstractBuilding on the socioemotional wealth theory, this study examines the influence of family ownership and corporate social responsibility (CSR) on trade credit. We argue that the intention to preserve family control, the preference for long-lasting relationships, and the desire to accumulate social capital lead family firms to opt for trade credit. Family firms’ peculiarities are also expected to condition the CSR-trade credit link. In addition, we account for the fact that some CSR practices are particularly aimed at external stakeholders. Our analyses rely on a sample of European listed firms from 2008 to 2020 and our empirical evidence confirms a positive effect of family ownership and CSR on trade credit. Going a step further, our results highlight the moderating role of family ownership in the relationship between CSR and firm’s access to trade credit. In fact, the positive effect of CSR on trade credit seems to be exclusively attributable to family firms. We also report that CSR policies oriented towards external stakeholders are linked to greater use of trade credit, with family firms explaining the positive impact of external CSR.
Regularized step directions in nonlinear conjugate gradient methods
Authors: Buhler Cassidy K. , Benson Hande Y. , Shanno David F. 
Published: September 2024

AbstractConjugate gradient minimization methods (CGM) and their accelerated variants are widely used. We focus on the use of cubic regularization to improve the CGM direction independent of the step length computation. In this paper, we propose the Hybrid Cubic Regularization of CGM, where regularized steps are used selectively. Using Shanno’s reformulation of CGM as a memoryless BFGS method, we derive new formulas for the regularized step direction. We show that the regularized step direction uses the same order of computational burden per iteration as its non-regularized version. Moreover, the Hybrid Cubic Regularization of CGM exhibits global convergence with fewer assumptions. In numerical experiments, the new step directions are shown to require fewer iteration counts, improve runtime, and reduce the need to reset the step direction. Overall, the Hybrid Cubic Regularization of CGM exhibits the same memoryless and matrix-free properties, while outperforming CGM as a memoryless BFGS method in iterations and runtime.
Optimal approaches in global warming mitigation and adaptation strategies at city scale
Authors: Gupta Aman , Shukla Anoop Kumar 
Published: September 2024

AbstractCase studies from global cities contribute to more focused analyses of global warming challenges and demonstrate the performance and effectiveness of mitigation and adaptation strategies to identify lessons about success at the city scale. The case studies were chosen to demonstrate aspects of the critical messages for action priorities in global warming mitigation and adaptation. This work focuses on best practices and initiatives for mitigation and adaptation approaches from developed and developing economies, including North American cities, European cities, Asian cities, and other global cities worldwide. The case studies were grouped to examine, identify, and emphasize important factors in various areas (e.g., local programs and alliances, governance, stakeholder engagement, community actions, and scientific research) that determined the success of adaptation strategies in various global cities. Many recent studies showcase mitigation approaches, particularly those relating to blue-green infrastructure and nature-based strategies. The case studies selected reflect vulnerable regions and demonstrate how increasing global warming significantly concerns individuals, societies, and their infrastructure. The selected studies include Amsterdam in Netherlands; Singapore, as a city in a garden; Boston in USA; Ahmedabad Heat Action Plan in India, aimed at implementing strategies with the objectives of climate adaptation planning; Copenhagen, as a coastal town, is more susceptible to flooding; Portland, the most progressive city in USA; Hamburg in Germany, one of the biggest harbours in Europe; and the 'Rain City Strategy', in Vancouver, Canada. Not all global cities respond the same way, but undertaking joint complex efforts helps mitigate the impacts.
Privacy-Preserving Computing Services for Encrypted Personal Data Through Streams Over Distributed Ledgers
Authors: Ballesteros-Rodríguez Alberto , Sánchez-Alonso Salvador , Sicilia-Urbán Miguel-Ángel 
Published: September 2024

AbstractThe growing adoption of wearables is driving the demand for personalized services that leverage unprocessed data, such as biometric and health information, to enhance user experiences and support through software applications. However, several existing use cases involving this information still prioritize traditional schemes, neglecting user privacy. Consequently, the transparency of data transmission paths and the potential for tampering remain ambiguous when users share data with service providers. In this paper, we propose the application of an Internet of Things device-focused distributed ledger as an underlying layer for the transmission of encrypted data using streams. Moreover, our proposal enables data recording for future events and the implementation of multi-subscriber models, allowing client information to be shared securely with different service providers. Through simulation experiments conducted on constrained devices, we demonstrate that our proposed framework efficiently transmits large ciphertexts through streams on a distributed ledger, overcoming the inherent limitations of such networks when dealing with substantial data volumes. Ultimately, the performance metrics presented prove that the proposed model is suitable for real-world applications requiring continuous data collection by wearables and subsequent transmission to service providers.
What investors want from whom: international organizations and the International Association for the Promotion and the Protection of Private Foreign Investments (APPI) 1958–1974
Authors: Batselé Filip , Hafner Nicolas 
Published: September 2024

AbstractUsing archival material from states, international organizations, and business actors, this paper explores how the Association for the Promotion and Protection of Private Foreign Investments (APPI), a transnational business interest association (BIA), liaised with different international institutions to lobby for better foreign investment protection. We zoom in on the United Nations, the Organization for Economic Co-operation and Development, and the World Bank to examine how APPI influenced the global institutional landscape during its heydays from 1958 until 1974. We show that business actors, particularly oil and banking corporations, created APPI as a nimble, efficient alliance that could move faster than existing BIAs. We further demonstrate how companies “forum shop” between different BIAs, and how APPI injected its ideas into the policymaking process, using the framework of the three faces of power. By shedding light on the role private business actors played in foreign investor protection, the paper contributes to a better understanding of the emergence of global economic governance in the second half of the 20th century.
Institutional care and education: circulation of knowledge about epilepsy in Sweden 1915–40
Authors: Ringarp Johanna 
Published: September 2024

AbstractThis article focuses on the circulation of knowledge about epilepsy in Sweden between 1915 and 1940. During the period medical research on epilepsy increased, which simultaneously brought a new degree of specialisation and distinction between branches of medicine. The aim of this article is to study the impact of new medical knowledge about epilepsy on the treatment and education of children with epilepsy in Sweden. In order to concretise the aim, the study focuses on the asylum Margarethahemmet. The key source material consists of Margarethahemmet’s annual reports and yearbooks. The minutes of the meetings of the Swedish General Association for the Care of the Feebleminded and Epileptic for the period 1915–1938 have been used as supplementary material. In order to trace the impact of medical discoveries on Margarethahemmet’s operations, contemporary scientific articles, mostly from Germany, have also been used. The article demonstrates how new research and new knowledge was sought internationally and nationally, to provide doctors and special teachers at the asylum with a proper knowledge about education, care and treatment for children with epilepsy. The increased understanding of the disease directly impacted the ability of a stigmatized group – people with epileptic disorder – to actively participate in society on the same terms as others.
The Collective Responsibilities of Science: Toward a Normative Framework
Authors: Politi Vincenzo 
Published: September 2024

AbstractScientists have the epistemic responsibility of producing knowledge. They also have the social responsibility of aligning their research with the needs and values of various societal stakeholders. Individual scientists may be left with no guidance on how to prioritize and carry these different responsibilities. As I will argue, however, the responsibilities of science can be harmonized at the collective level. Drawing from debates in moral philosophy, I will propose a theory of the collective responsibilities of science that accounts for the internal diversity of research groups and for their different responsibilities.
Celtic music, Shakespeare and fandom
Authors: Cyrus Cynthia J. 
Published: September 2024

AbstractWhy would Celtic women performers invoke a dead colonialist English poet in their airs and dance sets? This article provides close readings of Shakespearean elements in the musical compositions of gold- and platinum-selling artists Loreena McKennitt, Ensemble Galilei, and Méav. It assesses the archaicising musical language, structural reshapings, and ekphrastic elements as signals of ‘restitutional creativity’ and trans-temporal collaboration. It also analyses the reception of these works by fans and critics, responses replete with invocations of tradition and of timelessness. Like the fanfic-producing enthusiasts of Johnathan Pope's Shakespeare's Fans, the fans for Celtic music capitalise on their awareness of Shakespearean elements in this music to display personal expertise. In so doing, they enhance their own belonging within the Celtic music community.
The Greatest Rock and Soul Band in the World? The Rolling Stones, genre and race
Authors: Marshall Lee 
Published: September 2024

AbstractThe Rolling Stones played a core role in establishing the generic conventions of rock. A key ideological element of this was the band's reverential dedication to the blues and the importance of the blues to their musical development. However, what is much less recognised is the influence of soul on the band's sound. By looking at the band's repertoire, composition and performing style, this paper explores the influence that soul, particularly Southern soul, had on the band's formative years and argues that, in many ways, they have adopted the aesthetic conventions of soul, rather than rock, for the majority of their career. Rethinking The Stones’ style in this way may help us better understand their position in the rock canon, while also encouraging careful interrogation of the racialised division of rock and soul that emerged in the late 1960s.
Fixed-time control of teleoperation systems based on adaptive event-triggered communication and force estimators
Authors: Liu Xia , Wen Hui 
Published: September 2024

AbstractA fixed-time control strategy based on adaptive event-triggered communication and force estimators is proposed for a class of teleoperation systems with time-varying delays and limited bandwidth. Two force estimators are designed to estimate the operator force and environment force instead of force sensors. With the position, velocity, force estimate signals, and triggering error, an adaptive event-triggered scheme is designed, which automatically adjusts the triggering thresholds to reduce the access frequency of the communication network. With the state information transmitted at the moment of event triggering while considering the time-varying delays, a fixed-time sliding mode controller is designed to achieve the position and force tracking. The stability of the system and the convergence of tracking error within a fixed time are mathematically proved. Experimental results indicate that the control strategy can significantly reduce the information transmission, enhance the bandwidth utilization, and ensure the convergence of tracking error within a fixed time for teleoperation systems.
A study on path-planning algorithm for a multi-section continuum robot in confined multi-obstacle environments
Authors: Gao Guohua , Li Dongjian , Liu Kai , Ge Yuxin , Song Chunxu 
Published: September 2024

AbstractIn confined multi-obstacle environments, generating feasible paths for continuum robots is challenging due to the need to avoid obstacles while considering the kinematic limitations of the robot. This paper deals with the path-planning algorithm for continuum robots in confined multi-obstacle environments to prevent their over-deformation. By modifying the tree expansion process of the Rapidly-exploring Random Tree Star (RRT*) algorithm, a path-planning algorithm called the continuum-RRT* algorithm herein is proposed to achieve fewer iterations and faster convergence as well as generating desired paths that adhere to the kinematic limitations of the continuum robots. Then path planning and path tracking are implemented on a tendon-driven four-section continuum robot to validate the effectiveness of the path-planning algorithm. The path-planning results show that the path generated by the algorithm indeed has fewer transitions, and the path generated by the algorithm is closer to the optimal path that satisfies the kinematic limitations of the continuum robot. Furthermore, path-tracking experiments validate the successful navigation of the continuum robot along the algorithm-generated path, exhibiting an error range of 2.51%–3.91%. This attests to the effectiveness of the proposed algorithm in meeting the navigation requirements of continuum robots.
The Vision of the Holy Rood
Authors: Baker Aidan 
Published: September 2024

When Augustine, Columba and Aidan converted the English in the sixth and
seventh centuries, their devoted labours profoundly affected the national
literature. Despite the hazy general belief that English History and
Literature set off together in 1066 on their long march, England from before
the time of Alfred had had a great tradition of prose and verse. Indeed, in
the ninth century, she had a considerable body of prose hterature when other
languages had hardly even begun verse.
English poetry from the seventh century onwards gives abundant proof of
the ardour with which the Teutonic conquerors of Britain embraced the
religion of Christ; and in less than a century these once pagan invaders had
made their adopted land into a splendid home of Christianity. The Church's
influence on the English way of life was deep and wide. Churches were built,
monasteries established, and schools founded; no mean accomplishment in such
war-like days.
Explicit information and practice type can affect the L2 acquisition of plural marking
Authors: Lucas Matt 
Published: September 2024

AbstractPrevious research has indicated that explicit information (EI) about either the first language (L1) or second language (L2) along with task-essential practice can facilitate L2 learning (e.g., Fernández, 2008; McManus, 2022). However, little research has examined L1–L2 contrastive EI with L1/L2 practice. Targeting plural-marking accuracy, the present study sought to fill this gap by exposing 127 Japanese intermediate learners of English to six online treatment sessions across four conditions: (1) non-contrastive EI (pluralization rule explanation) + L2 practice, (2) contrastive EI + L2 practice, (3) as per Condition 2 + additional L1 practice (application of L2 pluralization to L1 through the Japanese suffix –tachi), and (4) using prepositions as a control, non-contrastive EI (prepositional rule explanation) + L2 practice. Accuracy was tested at pre/post/delayed intervals using an acceptability judgement task (reading) and a picture description task (writing). Statistical analyses demonstrated greater gains from the contrastive variables in the immediate post-test, with L1 practice appearing especially beneficial, despite improvements diminishing in the delayed post-test. Supplementary data from retroactive interviews and questionnaire responses indicated favourable orientations towards the intervention. The study advances understanding of how EI and practice type can be used to maximize L2 learning through web-based practices tailored to the nature of the learning problem.