bluuebunny/crossref_metadata_2025_split huggingface dataset:
Plakophilin 2 gene therapy prevents and rescues arrhythmogenic right ventricular cardiomyopathy in a mouse model harboring patient genetics
Authors: Bradford William H. , Zhang Jing , Gutierrez-Lara Erika J. , Liang Yan , Do Aryanne , Wang Tsui-Min , Nguyen Lena , Mataraarachchi Nirosh , Wang Jie , Gu Yusu , McCulloch Andrew , Peterson Kirk L. , Sheikh Farah 
Published: December 2023

AbstractArrhythmogenic right ventricular cardiomyopathy (ARVC) is a fatal genetic heart disease characterized by cardiac arrhythmias, in which fibrofatty deposition leads to heart failure, with no effective treatments. Plakophilin 2 (PKP2) is the most frequently mutated gene in ARVC, and although altered RNA splicing has been implicated, there are no models to study its effect and therapeutics. Here, we generate a mouse model harboring a PKP2 mutation (IVS10-1G>C) affecting RNA splicing, recapitulating ARVC features and sudden death starting at 4 weeks. Administering AAV-PKP2 gene therapy (adeno-associated viral therapy to drive cardiac expression of PKP2) to neonatal mice restored PKP2 protein levels, completely preventing cardiac desmosomal and pathological deficits associated with ARVC, ensuring 100% survival of mice up to 6 months. Late-stage AAV-PKP2 administration rescued desmosomal protein deficits and reduced pathological deficits including improved cardiac function in adult mice, resulting in 100% survival up to 4 months. We suggest that AAV-PKP2 gene therapy holds promise for circumventing ARVC associated with PKP2 mutations, including splice site mutations.
Structural and functional coupling in cross-linking uracil-DNA glycosylase UDGX
Authors: Liang Chuan , Yang Ye , Ning Ping , Chang Chenyan , Cao Weiguo 
Published: December 2023

Abstract
Enzymes in uracil-DNA glycosylase (UDG) superfamily are involved in removal of deaminated nucleobases such as uracil, methylcytosine derivatives such as formylcytosine and carboxylcytosine, and other base damage in DNA repair. UDGX is the latest addition of a new class to the UDG superfamily with a sporadic distribution in bacteria. UDGX type enzymes have a distinct biochemical property of cross-linking itself to the resulting AP site after uracil removal. Built on previous biochemical and structural analyses, this work comprehensively investigated the kinetic and enzymatic properties of Mycobacterium smegmatis UDGX. Kinetics and mutational analyses, coupled with structural information, defined the roles of E52, D56, D59, F65 of motif 1, H178 of motif 2 and N91, K94, R107 and H109 of motif 3 play in uracil excision and cross-linking. More importantly, a series of quantitative analyses underscored the structural coupling through inter-motif and intra-motif interactions and subsequent functional coupling of the uracil excision and cross-linking reactions. A catalytic model is proposed, which underlies this catalytic feature unique to UDGX type enzymes. This study offers new insight on the catalytic mechanism of UDGX and provides a unique example of enzyme evolution.
The Effect of Rating Method on Reliability of Judgments of Strain Across Populations
Authors: Sauder Cara L. , Kapsner-Smith Mara R. , Simmons Emily , Meyer Tanya , Doyle Philip C. , Eadie Tanya L. 
Published: December 2023

Purpose:
Variability in auditory-perceptual ratings of voice limits their utility, with the poorest reliability often noted for vocal strain. The purpose of this study was to determine whether an experimental method, called visual sort and rate (VSR), promoted stronger rater reliability than visual analog scale (VAS), for ratings of strain in two clinical populations: adductor laryngeal dystonia (ADLD) and vocal hyperfunction (VH).

Method:

Connected speech samples from speakers with ADLD and VH as well as age- and sex-matched controls were selected from a database. Fifteen inexperienced listeners rated strain for two speaker sets (25 ADLD speakers and five controls; 25 VH speakers and five controls) across four rating blocks: VAS-ADLD, VSR-ADLD, VAS-VH, and VSR-VH. For the VAS task, listeners rated each speaker for strain using a vertically oriented 100-mm VAS. For the VSR task, stimuli were distributed into sets of samples with a range of severities in each set. Listeners sorted and ranked samples for strain within each set, and final ratings were captured on a vertically oriented 100-mm VAS. Intrarater reliability (Pearson's
r
) and interrater variability (mean of the squared differences between a listener's ratings and group mean ratings) were compared across rating methods and populations using two repeated-measures analyses of variance.

Results:
Intrarater reliability of strain was significantly stronger when listeners used VSR compared to VAS; listeners also showed significantly better intrarater reliability in ADLD than VH. Listeners demonstrated significantly less interrater variability (better reliability) when using VSR compared to VAS. No significant effect of population or interactions was found between listeners for measures of interrater variability.

Conclusions:
VSR increases intrarater reliability for ratings of vocal strain in speakers with VH and ADLD. VSR decreases variability of auditory-perceptual judgments of strain between inexperienced listeners in these clinical populations. Future research should determine whether benefits of VSR extend to voice clinicians and/or clinical settings.
Nonlinear‐disturbance‐observer‐based predictive control for trajectory tracking of planar motors
Authors: Huang Su‐Dan , Xu Zhi‐Hui , Cao Guang‐Zhong , Wu Chao , He Jiangbiao 
Published: December 2023

AbstractTo improve the trajectory tracking performance of planar motors against disturbances, model predictive position control (MPPC) methods using the non‐linear disturbance observer (NDO) are proposed in this study. Based on the single‐axis dynamic model with disturbances, a single‐axis NDO is designed using an extended state observer approach. The designed NDO is expressed as a third‐order non‐linear state‐space equation in which the position error, velocity error, and lumped disturbance in the single axis are taken as the state variables. Two MPPC methods are developed based on the NDO. In the first MPPC, the disturbance is embedded into the prediction model using the NDO, and a controller is designed to minimise a quadratic cost function, which is established by applying the prediction model with disturbance. The output of the controller is the control action. In the second MPPC, a controller is used to minimise the quadratic cost function, which is built by employing the prediction model without disturbance. The sum of the output of the controller and the compensated disturbance estimated by the NDO is the control action. The comparative experiment is performed on a planar motor system self‐developed in the laboratory. The effectiveness of the proposed methods is verified via the experimental results.
Designing of an extract production protocol for industrial application of cell‐free protein synthesis technology: Building from a current best practice to a quality by design approach
Authors: Melinek Beatrice Judith , Tuck Jade , Probert Philip , Branton Harvey , Bracewell Daniel G. 
Published: December 2023

AbstractCell‐Free Protein Synthesis (CFPS) has, over the past decade, seen a substantial increase in interest from both academia and industry. Applications range from fundamental research, through high‐throughput screening to niche manufacture of therapeutic products. This review/perspective focuses on Quality Control in CFPS. The importance and difficulty of measuring the Raw Material Attributes (RMAs) of whole cell extract, such as constituent protein and metabolite concentrations, and of understanding and controlling these complicated enzymatic reactions is explored, for both centralised and distributed industrial production of biotherapeutics. It is suggested that a robust cell‐free extract production process should produce cell extract of consistent quality; however, demonstrating this is challenging without a full understanding of the RMAs and their interaction with reaction conditions and product. Lack of technology transfer and knowledge sharing is identified as a key limiting factor in the development of CFPS. The article draws upon the experiences of industrial process specialists, discussions within the Future Targeted Healthcare Manufacturing Hub Specialist Working Groups and evidence drawn from various sources to identify sources of process variation and to propose an initial guide towards systematisation of CFPS process development and reporting. These proposals include the development of small scale screening tools, consistent reporting of selected process parameters and analytics and application of industrial thinking and manufacturability to protocol development.
Integrated modelling of vacuum flashover development: Above‐surface processes and breakdown threshold analyses
Authors: Sun Guangyu , Yao Yitong , Yang Xiong , Guo Baohong , Zhang Shu , Wang Chao , Lian Ruhui , Song Baipeng , Mu Haibao , Zhang Guanjun 
Published: December 2023

AbstractThe flashover in vacuum is a rapid interfacial discharge across the insulator surface when subjected to high applied voltage. Here a theoretical model covering above‐surface processes is introduced. The model calculates the flashover threshold in vacuum, with revised secondary electron emission avalanche theory and improved desorbed neutral transport model. The model serves as the first part of an integrated flashover model, aiming for consistent treatment of both above‐surface and subsurface processes during the entire flashover development. The flashover threshold is obtained by combining the desorbed neutral pressure at given applied voltage and the gas breakdown criterion dictated by the Paschen's law. An analytical formula for threshold estimation containing physical parameters, and a simplified formula consisting of empirical coefficients are introduced, catering for both conceptual understanding and practical application. The derived formulae are validated by experimental data for a variety of insulator materials. The theory generalisation for non‐uniform electric field distribution is further discussed.
Health evaluation and dangerous reptile detection using a novel framework powered by the YOLO algorithm to design high‐content cellular imaging systems
Authors: Pandey Saroj Kumar , Kumar Ankit , Yadav Dhirendra Prasad , Sinha Anurag , Hassan Md. Mehedi , Singh N. K. , Bhatnagar Yash , Garg Namit 
Published: December 2023

AbstractThis novel approach in animal biology could revolutionize identifying endangered species, addressing the issue of misclassifying potentially harmful animals based solely on visual characteristics. Particularly impactful for farmers in agricultural fields, it aims to reduce the heightened risk of venomous animal attacks, ultimately improving safety. Due to a lack of accessible education, illiterate farmers are more susceptible to adopting superstitious beliefs, which tragically leads to fatal snakebites even when medical treatment is readily available. Furthermore, environmental factors can unexpectedly hold typically non‐threatening animals responsible for a large number of human deaths each year. However, the complexity of human recognition of these hazards has prompted the development of a novel design approach aimed at simplifying the process. Integration of the ResNet learning algorithm in conjunction with You Only Look Once (YOLOv5) within the framework is recommended to facilitate real‐time processing and improve accuracy. This combined approach not only speeds up animal recognition but also takes advantage of ResNet's deep learning capabilities. The first phase entails deploying YOLOv5 to detect the presence of snakes in the proposed study, achieving a remarkable 87% precision in snake detection thanks to the synergistic fusion of ResNet and YOLOv5.
ETXRE: Energy and delay efficient routing metric for RPL protocol and wireless sensor networks
Authors: Nait Abbou Aiman , Manner Jukka 
Published: December 2023

AbstractInternet of Things is an emerging paradigm based on interconnecting physical and virtual objects with each other and to the Internet. Most connected things fall into the category of constrained devices, with restricted resources (processing power, memory, and energy). These low‐power and lossy networks (LLNs) are known for their instability, high loss rates and low data rates, which makes routing one of the most challenging problems in low‐cost communications. A routing protocol for low‐power and lossy networks (RPL) is a proactive dynamic routing protocol based on IPv6. This protocol defines an objective function (OF) that utilises a set of metrics to select the best possible path to the destination. Minimum rank hysteresis objective function (MRHOF) and objective function zero (OF0) are the most basic OFs, where the first one selects the path to the sink based on the expected transmission count (ETX) metric, and OF0 is based on the hop count (HC). These two metrics prioritise either brute performance (i.e. ETX) or simplicity (i.e. HC). Therefore, using a single metric with an OF can either limit the performance or have an inefficient impact on load management and energy consumption. To overcome these challenges, a routing metric based on MRHOF OF which takes into consideration the link‐based routing metric (i.e. ETX) and node‐based metric (i.e. remaining energy) for route selection is provided. Expected transmission count remaining energy (ETXRE) is evaluated through 36 scenarios with different parameters. Preliminary results show that ETXRE outperforms ETX and RE in terms of end‐to‐end delay by an average of at least 17%, packet delay by 13% and consumes 10% less energy.
Kieferorthopädisches Behandlungskonzept zur Therapie der
bilateralen, diphyodontischen Inzisiven-Gemination bei gleichzeitiger
Eckzahnimpaktion – Ein Fallbericht
Authors: Wiechens Bernhard , Meyer-Marcotty Philipp 
Published: December 2023

Zusammenfassung
Hintergrund Dentale Anomalien sind durchschnittlich in jeder 5.
Hauptbehandlungskasuistik anzutreffen, allerdings zeigen sich verschiedene
Häufigkeiten und Graduierungen der Kategorien. Besonders in Kombination
verschiedener Anomalien imponieren schnell unikate Kasuistiken, denen nur mit
schwach belegten Empfehlungen begegnet werden kann.
Fallvorstellung Die 10-jährige Patientin stellte sich in der
Poliklinik für Kieferorthopädie aufgrund ihrer gestörten
Frontzahnästhetik vor. Diagnostisch konnte eine paarige diphyodontische
Gemination der zentralen Inzisivi und gleichzeitige Eckzahnimpaktion befundet
werden, die mittels Extraktion der Anomalie und Transplantation der zweiten
Prämolaren des Gegenkiefers, unter Zuhilfenahme einer
Multi-Bracket-Apparatur und skelettal-getragenen Führungsapparatur,
behandelt wurde.
Schlussfolgerungen Die Kasuistik konnte erfolgreich nach avisiertem
Behandlungskonzept therapiert werden. Die umfangreiche Behandlung machte den
enormen Stellenwert der interdisziplinären Versorgung deutlich und
konnte sich als brauchbare Therapiekonzeption für äquivalente
Fälle, bei gegebener fachlicher Infrastruktur, erweisen.
Cerebral Venous Thrombosis in Pediatric Age: Risk Factors and Prognosis
Authors: Pais-Cunha Inês , Almeida Ana I. , Curval Ana R. , Fonseca Jacinta , Melo Cláudia , Sampaio Mafalda , Sousa Raquel 
Published: December 2023

Abstract
Introduction Cerebral venous thrombosis (CVT) is a rare but potentially fatal disease in pediatric age with an important morbimortality. In adults several factors have been associated with worse outcomes, however there are still few studies in children. This study aims to identify risk factors associated with clinical manifestations and long-term sequelae in pediatric CVT.
Methods Retrospective analysis of pediatric inpatients admitted to a tertiary-care hospital due to CVT between 2008 and 2020.
Results Fifty-four children were included, 56% male, median age of 6.5 years (9 months–17.3 years). Permanent risk factors were identified in 13 patients (malignancy, 8; hematologic condition, 5) and transient risk factors in 47, including head and neck infections (57%) and head trauma (15%). Multiple venous sinuses involvement was present in 65% and the deep venous system was affected in four patients. Seventeen percent had intracranial hemorrhage and 9% cerebral infarction. Sixty-four percent of patients with multiple venous sinuses involvement presented with severe clinical manifestations: impaired consciousness, intracranial hypertension, acute symptomatic seizures or focal deficits. Regarding long-term prognosis, six patients had major sequelae: epilepsy (n = 3), sensory motor deficits (n = 2), and cognitive impairment (n = 3). Permanent risk factors were associated with severe clinical manifestations (p = 0.043). Cerebral infarction and intracranial hemorrhage were associated with major sequelae (p = 0.006 and p = 0.03, respectively, adjusted for age and sex).
Conclusion Permanent risk factors, involvement of multiple venous sinuses, intracranial hemorrhage, and cerebral infarction, were related to worse prognosis. Detection and early management of risk factors may limit CVT extension and reduce its morbimortality.
Linear Endoscopic Ultrasound Examination of the Biliary System and Its Clinical Applications
Authors: Chavan Radhika , Gandhi Chaiti , Patel Maitrey , Solanki Rushil , Rajput Sanjay 
Published: December 2023

AbstractEndoscopic ultrasound (EUS) examination of the biliary system plays pivotal role in pancreatobiliary studies. EUS offers a safe and noninvasive method of the biliary tract disorder evaluation. Although radial EUS provides a straightforward orientation, practicing biliary system examination with a curvilinear echoendoscope is advisable because of its added therapeutic benefits. Linear EUS may pose challenge in understanding the orientation and tracing nondilated bile duct in the beginning. However, adopting a systematic station-wise approach can help in comprehending the orientation and effectively tracing the entire bile duct. In this review, we have discussed linear EUS examination of the bile duct and gallbladder from various stations and its clinical applications.
The way I see the world, the way I envy others: a person-centered investigation of worldviews and the malicious and benign forms of envy among adolescents and adults
Authors: Ng Jacky C. K. , Chong Joanne Y. H. , Ng Hilary K. Y. 
Published: December 2023

AbstractPrevious research had extensively studied the consequences of dispositional malicious and benign envy, while relatively few studies examined its determinants. Although one’s worldviews have been proposed to shape the experience of malicious and benign envy, empirical studies directly investigating this notion are scarce. To address this gap, we adopted a person-centered approach to identify different individual profiles that underlie five generalized beliefs about the world, operationalized as five social axiom dimensions. We then examined how these profiles were associated with dispositional malicious and benign envy among both adolescents and working adults (N = 1248). As suggested by latent profile analysis, a 3-profile solution provided the best fit to the data in both groups. Two latent profiles (skeptical-pessimistic and hopeful-optimistic profiles) were similar across groups, while two distinct profiles (flexible and reserved profiles) were identified in adolescents and adults respectively. A series of comparisons indicated that people with different profiles experienced malicious and benign envy differently. In general, dispositional malicious envy was stronger among those in the skeptical-pessimistic profile, while dispositional benign envy was stronger among those in the hopeful-optimistic profile. Overall, our findings facilitate discussions on the similarities and differences in worldview profiles and experiences of envy across developmental groups.
Utilising locally based energy supplements in leucaena and corn stover diets to increase the average daily gain of male Bali cattle and the income of smallholder farmers
Authors: Hidayat Julian , Panjaitan Tanda , Dahlanuddin   , Harper Karen , Poppi Dennis 
Published: December 2023

Context Supplementing a leucaena-based diet with locally available energy sources is an important strategy to extend the use of leucaena, increase liveweight (LW) gain of Bali cattle and increase profit for the smallholder. Aims This study was conducted to evaluate the effect of various local energy supplements in leucaena-based diets on the LW gain of Bali cattle and income-over-feed costs (IOFC). Methods Forty male Bali cattle were divided into five treatment groups (n = 8) and each was fed one of the following diets: (A) control diet, including leucaena ad libitum, with corn stover at 0.5% DM LW and mineral mix, (B) control + cassava meal at 1.0% DM LW, (C) control + cassava peel at 1.0% DM LW, (D) control + cassava peel and corn grain mix at 1.0% DM LW or (E) control + commercial feed at 1.0% DM LW. The experiment was run for 140 days, which included a 20-day adaptation period. Parameters measured included intake, digestibility, imbibed-water intake, LW gain, rumen and faecal parameters, feed conversion and IOFC. Key results Supplementation of local energy sources in combination with leucaena and corn stover diets increased LW gain of male Bali cattle above that of the control diet (P < 0.05). The bulls receiving the cassava peel and corn mix supplement had the highest LW gain (0.57 ± 0.09 kg/day), which was associated with an increase in digestible organic-matter intake (DOMI). There were no treatment differences (P > 0.05) in the rumen parameters (rumen fluid pH, rumen ammonia-N concentration and volatile fatty acids) and all parameters were optimum for rumen digestion. Conclusions The addition of local energy supplements supplied at 1% of LW improved growth rate and extended the use of a limited amount of leucaena and provided a higher IOFC. Implications Replacing approximately 40% of leucaena with energy sources can have three benefits, including an increase in LW gain, an increased capacity of farmers to feed more cattle per hectare and an increased income per cattle being fattened. This can increase the production scale and subsequent farmer income, provided that the energy sources are available at affordable prices and obtainable.
Engagement in oral production
Authors: Phuong Thao Le Hong , Macalister John 
Published: December 2023

Abstract
Interaction is recognised as an important contributor to language learning but in many classrooms opportunities to interact meaningfully are limited. This situation can be addressed when teachers make decisions about adapting the materials they are working with. The result is likely to be learners who are more engaged in multiple ways and who therefore learn more effectively. This article reports on a two-phase investigation of English language learning in Grade 5 classes in Vietnam. In the first phase, twenty-one classes were observed to form an understanding of standard practices in these classes. In the second phase, a small, replicable adaptation of coursebook material was made and implemented with an intervention class for one semester. Three dimensions of engagement were investigated, and on each learners in the intervention class were considerably more engaged than in the comparison class, or in the first phase classes. This study demonstrates what it is possible to achieve through a small coursebook innovation.