bluuebunny/crossref_metadata_2025_split huggingface dataset:
Vision and sensorimotor defects associated with loss of Vps11 function in a zebrafish model of genetic leukoencephalopathy
Authors: Banerjee Shreya , Ranspach Lillian E. , Luo Xixia , Cianciolo Lauren T. , Fogerty Joseph , Perkins Brian D. , Thummel Ryan 
Published: March 2022

AbstractGenetic Leukoencephalopathies (gLEs) are heritable white matter disorders that cause progressive neurological abnormalities. A founder mutation in the human endolysosomal trafficking protein VPS11 has been identified in Ashkenazi Jewish patients manifesting classic gLE symptoms of hypomyelination, developmental delay, motor and systemic deficits. In this study, we characterized the visual and sensorimotor function of two zebrafish vps11 mutant lines: the previously reported vps11(plt), and a new vps11(−/−) null mutant line, using behavioral analysis to track larval motor responses to visual and acoustic stimuli. We found that mutant larvae from both vps11(plt) and vps11(−/−) lines were able to visually distinguish light and dark, but showed a progressive loss of a normal sensorimotor response to visual stimuli from 5 days post fertilization (dpf) to 7dpf. Additionally, optokinetic response analysis performed at 5dpf indicated that the mutants were significantly visually impaired. Both mutant lines also displayed a progressively lower sensorimotor response to a singular acoustic stimulus from 5-7dpf. Next, we tested the habituation response of the mutant lines to series of acoustic taps. We found both mutant lines habituated faster than their siblings, and that vps11(plt) mutants habituated faster than the vps11(−/−) mutants. Together, these data suggest that loss of Vps11 function results in progressive visual and sensorimotor abnormalities in the zebrafish vps11(plt) and vps11(−/−) mutant lines. This is the first study to characterize behavioral deficits in a vertebrate model of Vps11-dependent gLE. The mutants and behavioral assays described here could be a valuable model system in which to test potential pharmacological interventions for gLE.
Mitigating the susceptibility to intergranular corrosion of alloy 625 by friction-stir welding
Authors: Lemos Guilherme Vieira Braga , Farina Alexandre Bellegard , Piaggio Henrique , Bergmann Luciano , Ferreira Jane Zoppas , dos Santos Jorge Fernandez , Vander Voort George , Reguly Afonso 
Published: March 2022

AbstractIn this work, friction-stir welding (FSW) was employed to alloy 625 grade I (soft annealed) sheets. Therefore, solid-state based welding was undertaken with a tool rotational speed of 200 rpm and a welding speed of 1 mm/s. Microstructural features were analyzed by light optical and scanning electron microscopy (LOM and SEM). Moreover, microhardness measurements were performed. The susceptibility to intergranular corrosion was verified by the double-loop electrochemical potentiokinetic reactivation (DL-EPR) test. Complementary, intergranular corrosion was also evaluated by the ASTM G28 Method A. FSW promoted grain refinement, increased microhardness, and reduction in the degree of sensitization. Finally, the mean corrosion rate observed in the ASTM G28 Method A test was 0.4406 mm/year, which suggests a good weld quality.
A multicenter cohort study on the association between prehospital immobilization and functional outcome of patients following spinal injury in Asia
Authors: Chen Hsuan An , Hsu Shuo Ting , Shin Sang Do , Jamaluddin Sabariah Faizah , Son Do Ngoc , Hong Ki Jeong , Tanaka Hideharu , Sun Jen Tang , Chiang Wen Chu ,   The PATOS Clinical Research Network,   Participating Nation Investigators, Ramakrishnan T. V. , Jamaluddin Sabariah Faizah , Tanaka Hideharu , Velasco Bernadett , Hong Ki Jeong , Sun Jen Tang , Khruekarnchana Pairoj , LLC Saleh Fares , Son Do Ngoc ,   Participating Site Investigators, Rao Ramana , Abraham George P. , Ramakrishnan T. V. , Jamaluddin Sabariah Faizah , Mohidin Mohd Amin Bin , Saim Al-Hilmi , Kean Lim Chee , Anthonysamy Cecilia , Yssof Shah Jahan Din Mohd , Ji Kang Wen , Kheng Cheah Phee , Ali Shamila bt Mohamad , Ramanathan Periyanayaki , Yang Chia Boon , Chia Hon Woei , Hamad Hafidahwati Binti , Ismail Samsu Ambia , Abdullah Wan Rasydan B. Wan , Tanaka Hideharu , Kimura Akio , Velasco Bernadett , Gundran Carlos D. , Convocar Pauline , Sabarre Nerissa G. , Tiglao Patrick Joseph , Hong Ki Jeong , Song Kyoung Jun , Jeong Joo , Moon Sung Woo , Kim Joo-yeong , Cha Won Chul , Lee Seung Chul , Ahn Jae Yun , Lee Kang Hyeon , Yeom Seok Ran , Ryu Hyeon Ho , Kim Su Jin , Kim Sang Chul , Hu Ray-Heng , Sun Jen Tang , Wang Ruei-Fang , Hsieh Shang-Lin , Kao Wei-Fong , Riyapan Sattha , Tianwibool Parinya , Buaprasert Phudit , Akaraborworn Osaree , Al Sakaf Omer Ahmed , LLC Saleh Fares , Huy Le Bao , Son Do Ngoc , Van Dai Nguyen 
Published: March 2022

AbstractPrehospital spinal immobilization is a widely used procedure in the emergency medical service (EMS) system worldwide, while the incidence of patients with spinal injury (SI) is relatively low, and unnecessary prehospital spinal immobilization is associated with patient complications. This study aimed to determine the association between prehospital spine immobilization and favorable functional outcomes at hospital discharge among trauma patients with SI. We conducted a retrospective cohort study using the Pan-Asia Trauma Outcomes Study (PATOS) registry data from January 1, 2016, to November 30, 2018. A total of 759 patients with SI were enrolled from 43,752 trauma patients in the PATOS registry during the study period. The subjects had a median age of 58 years (Q1–Q3, 41–72), and 438 (57.7%) patients had prehospital spine immobilization. Overall, prehospital spinal immobilization was not associated with favorable functional outcomes at discharge in multivariable logistic regression (aOR 1.06; 95% CI 0.62–1.81, p = 0.826). However, in the subgroup of cervical SI, prehospital spinal immobilization was associated with favorable functional outcomes at discharge (aOR 3.14; 95% CI 1.04–9.50; p = 0.043). Therefore, we suggest that paramedics should be more careful when determining the presence of a cervical SI and should apply full spine immobilization if possible.
Thermal stability of Si/SiC/ta-C composite coatings and improvement of tribological properties through high-temperature annealing
Authors: Jang Young-Jun , Kim Jae-Il , Kim Won-seok , Kim Do Hyun , Kim Jongkuk 
Published: March 2022

AbstractWe report the structure, mechanical properties, thermal stability, and durability of Si/SiC/ta-C composite (Si–ta-C) coatings fabricated using simultaneous filtered cathodic vacuum arc deposition and direct current unbalanced magnetron sputtering. Si concentration of 1.25–6.04 at.% was achieved by increasing the unbalanced magnetron sputtering power from 25 to 175 W. Si addition provided functionality to the coating, such as heat resistance, while retaining the high hardness of ta-C coatings. The Si–ta-C coatings were stable up to 600 °C regardless of the Si content, while the coating containing 3.85 at.% Si was stable up to 700 °C. The friction behavior and mechanical properties were dependent on the coating film before and after annealing at 100–200 °C; however, annealing at 300–400 °C decreased disk wear and increased counterpart wear due to an increase in film hardness on account of an endothermic reaction that increased the number of Si–C bonds. This indicates that the basic hardness characteristics of the ta-C coating and the high-temperature structural change of the Si–ta-C coating are important for ensuring high-temperature durability. These characteristics were verified through the low coefficient of friction and wear rate of the 1.25 at.% Si–ta-C coating after annealing at 500 °C.
LIM domain-wide comprehensive virtual mutagenesis provides structural rationale for cardiomyopathy mutations in CSRP3
Authors: Chauhan Pankaj Kumar , Sowdhamini Ramanathan 
Published: March 2022

AbstractCardiomyopathies are a severe and chronic cardiovascular burden worldwide, affecting a large cohort in the general population. Cysteine and glycine-rich protein 3 (CSRP3) is one of key proteins implicated in dominant dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). In this study, we device a rapid in silico screening protocol that creates a mutational landscape map for all possible allowed and disallowed substitutions in the protein of interest. This map provides the structural and functional insights on the stability of LIM domains of CSRP3. Further, the sequence analysis delineates the eukaryotic CSRP3 protein orthologs which complements the mutational map, but provide limited information of amino acid exchanges. Next, we also evaluated the effect of HCM/DCM mutations on these domains. One of highly destabilising mutations—L44P (also disease causing) and a neutral mutation—L44M were further subjected to molecular dynamics (MD) simulations. The results establish that L44P substitution affects the LIM domain structure by altering secondary structure and due to loss of hydrophobic interaction with Phenylananine 35. The present study provides a useful perspective to our understanding of the role of mutations in the CSRP3 LIM domains and their evolution. This study provides a novel computational screening method for quick identification of key mutation sites for specific protein structures that can reduce the burden on experimental research.
Cranial stent position is independently associated with the development of TIPS dysfunction
Authors: Meyer Carsten , Paar Pérez Alba Maria , Chang Johannes , Sprinkart Alois Martin , Böhling Nina , Luu Andreas Minh , Kütting Daniel , Jansen Christian , Luetkens Julian , Bischoff Leon Marcel , Attenberger Ulrike , Strassburg Christian P. , Trebicka Jonel , Wolter Karsten , Praktiknjo Michael 
Published: March 2022

AbstractComplications of portal hypertension can be treated with transjugular intrahepatic portosystemic shunt (TIPS) in selected patients. TIPS dysfunction is a relevant clinical problem. This study investigated the prognostic value of two-dimensional (2D) TIPS geometry for the development of TIPS dysfunction. Three hundred and seven patients undergoing TIPS procedure between 2014 and 2019 were analyzed in this monocentric retrospective study. 2D angiograms from the patients with and without TIPS dysfunction were reviewed to determine geometric characteristics including insertion and curve angles and the location of the stent. Primary outcome was the development of TIPS dysfunction. A total of 70 patients developed TIPS dysfunction and were compared to the dysfunction-free (n = 237) patients. The position of the cranial stent end in the hepatic vein and the persistence of spontaneous portosystemic shunts were significantly associated with the development of TIPS dysfunction. Among significant parameters in univariable regression analysis (portal vein-pressure after TIPS, Child–Pugh Score before TIPS, MELD before TIPS and white blood cell count before TIPS), multivariable models showed cranial stent position (p = 0.027, HR 2.300, 95% CI 1.101–4.806) and SPSS embolization (p = 0.006, HR 0.319, 95% CI 0.140–0.725) as the only predictors of TIPS dysfunction. This monocentric study demonstrates that the position of the cranial stent end is independently associated with the development of TIPS dysfunction. The distance of the cranial stent end to the IVC at the time of TIPS placement should be less than 1 cm in 2D angiography.
Evaluation of a commercial ELISA as alternative to plaque reduction neutralization test to detect neutralizing antibodies against SARS-CoV-2
Authors: Hofmann Natalie , Grossegesse Marica , Neumann Markus , Schaade Lars , Nitsche Andreas 
Published: March 2022

AbstractHigh-throughput detection of neutralizing antibodies against SARS-CoV-2 presents a valuable tool for vaccine trials or investigations of population immunity. We evaluate the performance of the first commercial surrogate virus neutralization test (sVNT, GenScript Biotech) against SARS-CoV-2 plaque reduction neutralization test (PRNT) in convalescent and vaccinated individuals. We compare it to five other ELISAs, two of which are designed to detect neutralizing antibodies. In 491 pre-vaccination serum samples, sVNT missed 23.6% of PRNT-positive samples when using the manufacturer-recommended cutoff of 30% binding inhibition. Introducing an equivocal area between 15 and 35% maximized sensitivity and specificity against PRNT to 72.8–93.1% and 73.5–97.6%, respectively. The overall diagnostic performance of the other ELISAs for neutralizing antibodies was below that of sVNT. Vaccinated individuals exhibited higher antibody titers by PRNT (median 119.8, IQR 56.7–160) and binding inhibition by sVNT (median 95.7, IQR 88.1–96.8) than convalescent patients (median 49.1, IQR 20–62; median 52.9, IQR 31.2–76.2). GenScript sVNT is suitable to screen for SARS-CoV-2-neutralizing antibodies; however, to obtain accurate results, confirmatory testing by PRNT in a equivocal area is required. This equivocal area may require adaptation for use in vaccinated individuals, due to higher antibody titers.
Emergence of active turbulence in microswimmer suspensions due to active hydrodynamic stress and volume exclusion
Authors: Qi Kai , Westphal Elmar , Gompper Gerhard , Winkler Roland G. 
Published: March 2022

AbstractMicroswimmers exhibit an intriguing, highly-dynamic collective motion with large-scale swirling and streaming patterns, denoted as active turbulence – reminiscent of classical high-Reynolds-number hydrodynamic turbulence. Various experimental, numerical, and theoretical approaches have been applied to elucidate similarities and differences of inertial hydrodynamic and active turbulence. We use squirmers embedded in a mesoscale fluid, modeled by the multiparticle collision dynamics (MPC) approach, to explore the collective behavior of bacteria-type microswimmers. Our model includes the active hydrodynamic stress generated by propulsion, and a rotlet dipole characteristic for flagellated bacteria. We find emergent clusters, activity-induced phase separation, and swarming behavior, depending on density, active stress, and the rotlet dipole strength. The analysis of the squirmer dynamics in the swarming phase yields Kolomogorov-Kraichnan-type hydrodynamic turbulence and energy spectra for sufficiently high concentrations and a strong rotlet dipole. This emphasizes the paramount importance of the hydrodynamic flow field for swarming motility and bacterial turbulence.
A consistent picture of excitations in cubic BaSnO3 revealed by combining theory and experiment
Authors: Aggoune Wahib , Eljarrat Alberto , Nabok Dmitrii , Irmscher Klaus , Zupancic Martina , Galazka Zbigniew , Albrecht Martin , Koch Christoph , Draxl Claudia 
Published: March 2022

AbstractAmong the transparent conducting oxides, the perovskite barium stannate is most promising for various electronic applications due to its outstanding carrier mobility achieved at room temperature. However, most of its important characteristics, such as band gaps, effective masses, and absorption edge, remain controversial. Here, we provide a fully consistent picture by combining state-of-the-art ab initio methodology with forefront electron energy-loss spectroscopy and optical absorption measurements. Valence electron energy-loss spectra, featuring signals originating from band gap transitions, are acquired on defect-free sample regions of a BaSnO3 single crystal. These high-energy-resolution measurements are able to capture also very weak excitations below the optical gap, attributed to indirect transitions. By temperature-dependent optical absorption measurements, we assess band-gap renormalization effects induced by electron-phonon coupling. Overall, we find for the effective electronic mass, the direct and the indirect gap, the optical gap, as well as the absorption onsets and spectra, excellent agreement between both experimental techniques and the theoretical many-body results, supporting also the picture of a phonon-mediated mechanism where indirect transitions are activated by phonon-induced symmetry lowering. This work demonstrates a fruitful connection between different high-level theoretical and experimental methods for exploring the characteristics of advanced materials.
2470 million-year-old banded iron formation reveals a climatic oscillation consistent with the Gleissberg solar cycle
Authors: Ding Anyang , Hofmann Axel , Li Yi-Liang 
Published: March 2022

AbstractModern-day solar cycles due to the solar magnetic field oscillation are well recognized. Owing to the response of Earth’s climate to solar activity fluctuation, solar cycles in the Phanerozoic Eon have been recorded by laminites and fossil tree rings. However, the existence of magnetic cycles within the Sun younger than 3100 million-year-old is still unknown. The deposition of Precambrian banded iron formations (BIFs) reflects the primary productivity of the early ferruginous oceans and is coupled to climatic fluctuations. Here we apply synchrotron-radiation-based µ-XRF with a 20 µm interval on a 60 mm long, 2470 million-year-old BIF from the Kuruman Formation, South Africa. Our spectral analyses of multiple elemental concentration series reveal prominent and consistent 80-year cyclicity, which is best explained by the Gleissberg solar cycle. We hypothesize that solar cycles have already modulated the early Paleoproterozoic climate mainly through high energy irradiance coupled solar radiative forcing oscillation and the solar magnetic field controlled galactic cosmic ray fluctuation. Our result provides the record of solar magnetic field oscillations with stable and close-to-present cycle durations at least since the beginning of the Paleoproterozoic Era.
Economic decision‐making systems in critical times: The case of ‘Bolsa Familia’ in Brazil
Authors: Pereira Junior Alfredo , Moroni Juliana 
Published: March 2022

AbstractKahneman's theory of two systems assumes that human decision making in Economy is based on two cognitive systems, one that is automatic, intuitive and mostly unconscious, and one that is reflexive, rational and fully conscious. The authors consider Kahneman’s approach incomplete and limited in accounting for the creativity of embodied agents grasping the opportunities afforded by physical and social environments. This limitation leads us to argue for the existence of a third system in decision making in Economy, the creative intuition based on direct perception of affordances, addressing not only the dispositions and goals of agents but also the social and environmental responsibility of corporations and governments. The authors argue that the third decision‐making system the authors propose implies a concept of intuition that is different from the type of association process discussed by Morewedge and Kahneman [2] and gives an example of the third system operation, the Bolsa Família in Brazil, and finding new options for the funding of similar programs.
Hosting a community‐based local electricity market in a residential network
Authors: Saif Aziz , Khadem Shafi K. , Conlon Michael , Norton Brian 
Published: March 2022

AbstractThis paper presents the potential of building a local electricity market (LEM) to boost the deployment of the local energy communities, centred around active customers with distributed energy resources (DERs). To conduct a comprehensive and detailed study on different cases with reduced computational burdens, this paper adopts a simplified modelling approach where the market and network model simulations are performed in a cascaded, decoupled fashion. This allows achieving the optimal LEM output for the energy community with different DER assets that are not bounded by the network constraints. The investigation involves quantifying the benefits brought by LEM to energy communities by tapping the flexibility associated with trading inside the energy community. Moreover, it presents the influence of different types of DERs (mainly photovoltaics (PV) and energy storage (ES)) in customers' premises on the outcome of the LEM. The LEM demonstrates successfully the reduction of cost associated with the energy purchased from the energy retailer and maximises the consumption of locally generated clean electricity. Among the studied DER portfolios, the combination of PV and ES solution shows the highest economic potential but deteriorates the voltage profiles and shows high active power loss in the winter month among all the cases examined.
Religiosität und psychische Gesundheit
Authors: Steffens Markus , Neddens Anne-Katharina 
Published: March 2022

ZUSAMMENFASSUNGReligiosität kann für den Menschen eine weit über Gesundheitsfragen hinausgehende, ganzheitliche Bedeutung haben. In der Internationalen Klassifikation der Funktionsfähigkeit, Behinderung und Gesundheit (ICF) wird sie im Sinne der funktionalen Gesundheit in mehreren Säulen berücksichtigt. Religiosität zeigt häufiger eine positive Korrelation zur psychischen Gesundheit, aber auch nicht unwesentlich eine negative. Sie wird als Schutzfaktor mit verstärkender Wirkung weiterer personaler und sozialer, protektiver Faktoren diskutiert. Dem Faktor Bindungssicherheit kommt eine besondere Bedeutung zu. Die Moderation zwischen Risiko und Schutz scheint abhängig von intrinsischer/extrinsischer Motivation, Zentralität der Religiosität, wahrgenommener Gottesbeziehung, sozialen Erfahrungen in der religiösen Gemeinschaft und vom religiösen Coping-Stil.
Vorteile eines universellen frühkindlichen Screenings auf die
familiäre Hypercholesterinämie in Deutschland
Authors: Paetow Ulrich , Kordonouri Olga , Schwab Karl Otfried 
Published: March 2022

Zusammenfassung
Hintergrund Kardiovaskuläre Ereignisse sind die wichtigste
Mortalitätsursache in der westlichen Welt. Die familiäre
Hypercholesterinämie (FH) zeichnet sich durch erhöhte
low-density Lipoprotein-Cholesterin- und Gesamtcholesterin-Spiegel im peripheren
Blut aus. Die FH beruht zumeist auf einer genetischen Veränderung im
LDL-Rezeptorgen und gehört zu den häufigsten monogenen
Erkrankungen. Bei der homozygoten Form ist die Mortalität aufgrund
kardiovaskulärer Ereignisse im Kindes- und Jugendalter extrem hoch. Auch
die heterozygote Form der FH führt unbehandelt früh zu
kardiovaskulären Ereignissen und sollte frühzeitig
diagnostiziert und behandelt werden, um eine Atherosklerose zu vermeiden. Die
präventive und sichere Anwendung einer medikamentösen
Lipidsenkung konnte für Kinder gezeigt werden.
Methode Literatursuche in Cochrane library, Medline, NCBI databases.
Ergebnisse Anders als in europäischen Nachbarländern wurde
in Deutschland im Kindesalter kein universelles Screening auf diese
häufige Erkrankung etabliert, obwohl dies in den Leitlinien gefordert
wird. In der vorliegenden Arbeit wird die Literatur untersucht hinsichtlich der
Kausalität der Erkrankung, der Therapie im Kindesalter sowie
hinsichtlich des Zutreffens klassischer und modifizierter Kriterien für
das Durchführen eines vorgeschlagenen universellen FH-Screenings zum 5.
Geburtstag mit anschließendem reversen Kaskadenscreening bei
Verwandten.
Schlussfolgerung Die Autoren plädieren für die
Einführung eines universellen FH-Screenings in Deutschland zur
Vorsorgeuntersuchung U9 mit 5 Jahren. Eine Analyse anhand des Goldstandards
klassischer Kriterien nach Wilson und Jungner sowie anhand im zeitlichen Kontext
angepasster Kriterien zeigt die Vorteile eines universellen
frühkindlichen FH-Screening. Dieses kann präventiv das
kardiovaskuläre Risiko absenken, das Fortschreiten der Atherosklerose
verlangsamen und zu niedrigeren Inzidenzen von Schlaganfall und Herzinfarkt im
Erwachsenenalter beitragen. Neben den diagnostizierten FH-Kindern können
auch deren betroffene Verwandte erkannt werden.