bluuebunny/crossref_metadata_2025_split huggingface dataset:
Familial Cerebellar Ataxia and Amyotrophic Lateral Sclerosis/Frontotemporal Dementia with DAB1 and C9ORF72 Repeat Expansions: An 18‐Year Study
Authors: Rosenbohm Angela , Pott Hendrik , Thomsen Mirja , Rafehi Haloom , Kaya Sabine , Szymczak Silke , Volk Alexander E. , Mueller Kathrin , Silveira Isabel , Weishaupt Jochen H. , Tönnies Holger , Seibler Philip , Zschiedrich Katja , Schaake Susen , Westenberger Ana , Zühlke Christine , Depienne Christel , Trinh Joanne , Ludolph Albert C. , Klein Christine , Bahlo Melanie , Lohmann Katja 
Published: September 2022

ABSTRACTBackgroundCoding and noncoding repeat expansions are an important cause of neurodegenerative diseases.ObjectiveThis study determined the clinical and genetic features of a large German family that has been followed for almost 2 decades with an autosomal dominantly inherited spinocerebellar ataxia (SCA) and independent co‐occurrence of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).MethodsWe carried out clinical examinations and telephone interviews, reviewed medical records, and performed magnetic resonance imaging and positron emission tomography scans of all available family members. Comprehensive genetic investigations included linkage analysis, short‐read genome sequencing, long‐read sequencing, repeat‐primed polymerase chain reaction, and Southern blotting.ResultsThe family comprises 118 members across seven generations, 30 of whom were definitely and five possibly affected. In this family, two different pathogenic mutations were found, a heterozygous repeat expansion in C9ORF72 in four patients with ALS/FTD and a heterozygous repeat expansion in DAB1 in at least nine patients with SCA, leading to a diagnosis of DAB1‐related ataxia (ATX‐DAB1; SCA37). One patient was affected by ALS and SCA and carried both repeat expansions. The repeat in DAB1 had the same configuration but was larger than those previously described ([ATTTT]≈75[ATTTC]≈40‐100[ATTTT]≈415). Clinical features in patients with SCA included spinocerebellar symptoms, sometimes accompanied by additional ophthalmoplegia, vertical nystagmus, tremor, sensory deficits, and dystonia. After several decades, some of these patients suffered from cognitive decline and one from additional nonprogressive lower motor neuron affection.ConclusionWe demonstrate genetic and clinical findings during an 18‐year period in a unique family carrying two different pathogenic repeat expansions, providing novel insights into their genotypic and phenotypic spectrums. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
Implementation of deep learning in drug design
Authors: Yang Bo , Li Kan , Zhong Xiuqin , Zou Jun 
Published: September 2022

AbstractThe field of deep learning has witnessed dramatic and rapid progress in the past several years, largely driven by the availability of massive datasets and increased computational power. Although promising advances have been made, the implementation of deep learning in drug design is still in the vigorously developing stage. Here we summarize the frontiers and emerging applications of deep learning in drug design. We provide the background of deep learning and the architecture of several important deep neural networks for drug design and discovery, including convolutional neural networks, recurrent neural networks, transformer, deep auto‐encoder, generative adversarial networks, graph neural networks, and flow‐based neural networks. We give some examples of tasks for which these different deep neural networks are suitable. The successful employment of deep learning in drug design and discussing its further opportunities are reviewed, such as molecular representation, drug‐likeness prediction, de novo molecular generation, ligand‐protein interaction prediction, reaction, and retrosynthetic prediction. Current challenges and further possibilities are proposed to help accelerate the progress of the field, such as model interpretation, the requirement for large quantities of training datasets, hyperparameters tuning, and open source.
A holistic NMR framework to understand environmental impact: Examining the impacts of superparamagnetic iron oxide nanoparticles (SPIONs) in Daphnia magna via imaging, spectroscopy, and metabolomics
Authors: Jenne Amy , Soong Ronald , Gruschke Oliver , Bastawrous Monica , Monks Patricia , Moloney Cara , Brougham Dermot F. , Busse Falko , Bermel Wolfgang , Courtier‐Murias Denis , Wu Bing , Simpson Andre 
Published: September 2022

AbstractSuperparamagnetic iron oxide nanoparticles (SPIONs) are a contaminant of emerging interest, often used in the medical field as an imaging contrast agent, with additional uses in wastewater treatment and as food additives. Although the use of SPIONs is increasing, little research has been conducted on the toxic impacts to living organisms beyond traditional lethal concentration endpoints. Daphnia magna are model organisms for aquatic toxicity testing with a well understood metabolome and high sensitivity to SPIONs. Thus, as environmental concentrations continue to increase, it is becoming critical to understand their sub‐lethal toxicity. Due to the paramagnetic nature of SPIONs, a range of potential nuclear magnetic resonance spectroscopy (NMR) experiments are possible, offering the potential to probe the physical location (via imaging), binding (via relaxation weighted spectroscopy), and the biochemical pathways impacted (via in vivo metabolomics). Results indicate binding to carbohydrates, likely chitin in the exoskeleton, along with a decrease in energy metabolites and specific biomarkers of oxidative stress. The holistic NMR framework used here helps provide a more comprehensive understanding of SPIONs impacts on D. magna and showcases NMR's versatility in providing physical, chemical, and biochemical insights.
Influence of thermophoretic diffusion of nanoparticles with Joule heating in flow of Maxwell nanofluid
Authors: Irfan Muhammad 
Published: September 2022

AbstractThe aspects of activation energy in magnetized Maxwell nanofluid flow with Brownian movement and thermophoretic diffusion have been elaborated here. Furthermore, Joule heating, variable conductivity and chemical reaction are scrutinized. The Buongiorno nanofluid thought is ratify to incorporate the importance of thermophoretic and Brownian diffusion. The attained ODEs have been solved via homotopic algorithm. The performance of operational variables is inspected. The Maxwell temperature field for Eckert number and variable conductivity factor have similar trend. The fluid concentration exaggerates for activation energy and decelerates for Brownian motion parameter. Furthermore, the brilliant outcomes attained and associated with possible existing prose accurately.
The ecosystem services framework in archaeology (and vice versa)
Authors: Katz Ofir 
Published: September 2022

Abstract

Economics, ecology and archaeology study various aspects of resource utilisation and mobilisation, differing in the studied systems, objects and currencies. However, the three disciplines have developed mostly independently, resulting in limited dialogue among them. Emergent fields such as ecological economics and environmental archaeology are now linking the three disciplines and promoting dialogue among them, but a theoretical framework that links all three disciplines at once is missing.

I propose that ecosystem services (ES) can serve as such a theoretical framework. Moreover, after an ES‐centred framework establishes, it will be capable of further evolving – independently of ES—into a unified superdiscipline, relieving boundaries among disciplines.

To demonstrate this potential, I present some examples of archaeology‐ES linkages, relating to the past, present and future. I show, in general, how archaeology studies past ES and informs us on current ES, as well as how ES benefit archaeological practice. Thus, I demonstrate the strong interface between archaeology and ES, and how it can promote the dialogue among the three disciplines, provide them with new practical tools, and resolve theoretical issues as the sustainability of ancient societies and anthropocentricity and monetisation of ES.

Read the free Plain Language Summary for this article on the Journal blog.
Two‐way shape memory behavior of styrene‐based bilayer shape memory polymer plate
Authors: Du Haiyang , Yao Yongtao , Zhou Xiaoli , Zhao Yajun 
Published: September 2022

AbstractIn this work, a bilayer shape memory polymer (SMP) composite plate with two‐way shape memory behavior is simulated, in which two types of styrene‐based SMPs with well‐separated glass transition temperatures are assembled in parallel. The finite element (FE) software ABAQUS is selected to exhibit the two‐way shape memory effect during the shape recovery step and the Generalized Maxwell Model with the WLF equation is applied to characterize the temperature‐dependent properties of the SMP bilayer plates. The effect factors of axial predeformation, thermal expansion coefficient and plate thickness are all considered for the two‐way shape memory behavior of the styrene‐based bilayer SMP plate. After that, a smart gripper composed of four SMP composite plates is proposed to realize grabbing and releasing functions for one‐step and staged heating recovery. The FE results provide some necessary theoretical guidelines for future soft smart structural designs and optimization.
ALARP demonstration in management of change using quantitative Bowtie analysis risk assessment tool for an offshore gas platform
Authors: Abdul Aziz Mohammad Afif , Md Said Mohamad Syazarudin 
Published: September 2022

AbstractIn this study, a framework for a quantitative Bowtie analysis based on the layer of protection analysis (LOPA) approach in the management of change (MOC) process flow was developed. The proposed tool can be used as a quantitative risk analysis method in supporting decisions when layers of protection or safeguards are unavailable. The proposed tool can quantify the frequency of the causes of a particular hazard via Fault Tree Analysis and identify the multiple consequences of the impact of the hazards with quantitative end events for risk analysis. The practicality of the proposed tool was demonstrated in a selected case study from the MOC database from Company A's offshore gas platform, where the consequences and impacts of the hazards to the people, environment, assets, and reputation were assessed. The LOPA quantification was introduced into the Bowtie diagram using a reliability database to determine the failure rates and probability of failure of the affected equipment. The risk tolerance level was subsequently determined. The outputs of risk analysis from the proposed quantitative Bowtie were then compared with the previous qualitative risk evaluation of the selected case study. Potential opportunities for further use of the proposed quantitative Bowtie tool were recommended.
Loureirin C ameliorates ischemia and reperfusion injury in rats by inhibiting the activation of the TLR4/NF‐κB pathway and promoting TLR4 degradation
Authors: Xu Jikai , Liu Jingyu , Li Qing , Mi Yan , Zhou Di , Wang Jian , Chen Gang , Liang Dong , Li Ning , Hou Yue 
Published: September 2022

AbstractIschemic stroke is a leading cause of death and disability worldwide. Post‐ischemia, microglia respond immediately to the alternations in neuronal activity and mediate inflammation. Toll‐like receptor 4 (TLR4) plays a key role in this phenomenon. To explore the effect of loureirin C, an effective compound from Chinese Dragon's blood, on ischemic stroke, Sprague–Dawley rats were subjected to middle cerebral artery occlusion/reperfusion (MCAO/R) with/without intragastric administration of loureirin C (7, 14, and 28 mg/kg). Loureirin C alleviated MCAO/R‐induced brain impairment evaluated by neurological scores (p < 0.001), brain water content (p < 0.001), and cerebral infarct volume (p = 0.001). The neuroprotective (p < 0.001) and inhibitory effects on microglial activation (p < 0.001) of loureirin C were revealed by immunofluorescence. Rescue studies with TLR4 overexpression in BV‐2 microglia showed that the antiinflammatory effect of loureirin C was attributable to the inhibition of TLR4 protein expression. Moreover, co‐immunoprecipitation assays showed that the binding of Triad3A, an E3 ubiquitin ligase of TLR4, was increased by loureirin C (p = 0.003). Our study demonstrates that loureirin C could be a promising therapeutic agent for the management of ischemic stroke by inhibiting microglial activation, potentially by Triad3A‐mediated promotion of TLR4 ubiquitination and degradation.
Imidazole Promoted Efficient Anomerization of β‐D‐Glucose Pentaacetate in Solid State and Reaction Mechanism
Authors: Wang Meifeng , Zhang Liyin , Li Yiqun , Gu Liuqun 
Published: September 2022

AbstractAnomerization of glycosides was rarely performed under basic condition. Here, an imidazole promoted anomerization of β‐D‐glucose pentaacetate in solid state at room temperature was discovered. This unprecedent anomerization occurred after simple premixing and reaction proceeded continuously in solid state to full conversion without stirring or mechanomixing. Full conversion of all β‐forms in the mixture (α:β=2.2 : 1) into α‐forms only was demonstrated at 1 gram scale. A proposed mechanism involved with inter/intramolecular acyl transfer promoted by imidazoles in a concerted manner may promote the discovery of more new transformations in solid state.
Nature‐Inspired High Temperature Scale‐Resistant Slippery Lubricant‐Induced Porous Surfaces (HTS‐SLIPS)
Authors: Yao Xiaoxue , Lin Wenzhu , Wang Mingmei , Wang Steven 
Published: September 2022

AbstractScale formation is a longstanding and unresolved problem in a number of fields, including power production, petroleum exploration, thermal desalination, and construction. Herein, a high‐temperature scale‐resistant slippery lubricant‐induced surface (HTS‐SLIPS) is developed by one‐step electrodeposition and lubricant infusion. The fractal cauliflower‐like morphology with lubricant oil is conducive to forming an ultralow contact angle hysteresis of ≈1°. The 10‐d real‐world boiling trial indicates that by replacing the uncoated surface with HTS‐SLIPS, the reduction in scale mass is greater than 200% because of the low surface free energy (4.3 mJ m−2) and outstanding smoothness (Ra = 41 ± 8 nm) of HTS‐SLIPS. Thanks to the scale retardation, the bubble departure frequency of HTS‐SLIPS is eightfold higher than that of uncoated surfaces, signifying superior heat transfer efficiency. In these demonstrations, HTS‐SLIPS coated spiral tube exhibits better flowability and lower pressure drop than the uncoated one. In addition, favorable compatibility between HTS‐SLIPS and mechanical vibration is experimentally verified to strengthen the descaling of SLIPS synergistically. It is anticipated that the simple and scalable coating fabrication approach will be applicable in numerous industrial high‐temperature processes where scale formation is encountered.
Amplifying Dendritic Cell Activation by Bioinspired Nanometal Organic Frameworks for Synergistic Sonoimmunotherapy
Authors: Lu Zhixiang , Bai Shuang , Jiang Yonghe , Wu Shuaiying , Xu Dazhuang , Zhang Jianzhong , Peng Xuqi , Zhang Hongrui , Shi Yesi , Liu Gang 
Published: September 2022

AbstractDespite recent advancements of sonodynamic therapy (SDT) in cancer immunotherapy, challenges have yet to be surmounted to further boost its immunotherapeutic efficacy due to the low‐level tumor antigens presentation of dendritic cells (DCs). Cell membrane camouflaged‐nanoparticles can integrate the neoantigens of the cancer cell membrane with the multifunctionalities of synthetic nanocores. Herein, sono‐responsive nanoparticles coated with DC‐targeted antibody chimeric cancer cell membrane are investigated for multimodal therapy. The nanometal organic frameworks (MOFs) that respond to ultrasound are loaded successfully inside the vesicles displaying an anti‐DEC205 antibody. The anti‐DEC205 chimeric vesicles can directly target and activate DCs, promote tumor antigens cross‐presentation, and then produce a cascade amplified T‐cell immune response. Upon deep tissue‐penetrating sonication, AMR‐MOF@AuPt generates large amounts of reactive oxygen species that directly kill cancer cells, further initiating an anti‐cancer T cell immune response. Such synergistic sono‐immunotherapies effectually inhibit tumor growth and induce strong systemic and long‐term immune memory against cancer recurrence and distant metastasis. The authors findings provide DCs and tumor cells of a dual active‐targeting cell membrane‐coated sono‐immunotherapeutic nanoplatform for cancer therapy.
Distinguishing Ultrafast Energy Transfer in Atomically Thin MoS2/WS2 Heterostructures
Authors: Zeng Yan , Dai Wei , Ma Rundong , Li Zhe , Ou Zhenwei , Wang Cheng , Yu Yiling , Zhu Tong , Liu Xiaoze , Wang Ti , Xu Hongxing 
Published: September 2022

AbstractVan der Waals semiconducting heterostructures, known as stacks of atomically thin transition‐metal dichalcogenide (TMD) layers, have recently been reported as new quantum materials with fascinating optoelectronic properties and novel functionalities. These discoveries are significantly related to the interfacial carrier dynamics of the excited states. Carrier dynamics have been reported to be predominantly driven by the ultrafast charge transfer (CT) process; however, the energy transfer (ET) process remains elusive. Herein, the ET process in MoS2/WS2 heterostructures via transient absorption microscopy is reported. By analyzing the ultrafast dynamics using various MoS2/WS2 interfaces, an ET rate of ≈240 fs is obtain, which is not trivial to the CT process. This study elucidates the role of the ET process in interfacial carrier dynamics and provides guidance for engineering interfaces for optoelectronic and quantum applications of TMD heterostructures.
Waterbody status of upper Ganga basin and its major tributaries using enviro‐assessment techniques
Authors: Sharma Arvind Kumar , Malik Davendra Singh , Kamboj Vishal , Sharma Amit Kumar 
Published: September 2022

AbstractIn the past two decades, freshwater bodies and their water quality have deteriorated rapidly due to several natural and anthropogenic causes, as well as rapid urbanization and industrialization. Using environmental assessment tools, eight sampling stations were used to assess the water quality of the upper Ganga and its major headstreams between January 2019 and December 2021 and to assess the impact of human‐led activities on the environment and the suitability of the catchment basin to perpetuate aquatic ecological diversity. During the current study period, water quality indices such as the Water Quality Index (WQI), Nemerow Pollution Index (NPI), and the Overall index of pollution (OIP) were employed to assess water quality. Twelve physicochemical parameters were examined using appropriate techniques and then compared to international standards. A small increase was observed in some physicochemical parameters, such as pH, Turbidity, Dissolved Oxygen, and Biological Oxygen Demand, at sampling stations 7 and 8. In the upper Ganga basin, all three indices (WQI, NPI, and OIP) accurately recorded numerous natural and human events at the selected sample sites. The water quality demonstrated that aquatic species were abundant. The water quality at some sample stations was also negatively affected by human activities, such as riverbed mining. However, these types of activities must be monitored and assessed.
Einleitung
Authors: Kisch Tobias 
Published: September 2022

Zusammenfassung
Derzeitige Vergütungssysteme erlauben es kaum Sektorengrenzen aufzubrechen. Es existieren kaum Anreize zur Ambulantisierung und Ergebnisqualität. Der Reinerlös chirurgischer Einzelpraxen sinkt seit Jahren. Die Anzahl ambulanter Operationen nimmt dementsprechend ab. Ob die Besondere Versorgung in der Plastischen Chirurgie und Handchirurgie den Herausforderungen begegnen kann, soll in dieser Evaluation überprüft werden.
Ergebnisse der Projektrecherche und der empirischen Studie
Authors: Kisch Tobias 
Published: September 2022

Zusammenfassung
Die Ergebnisse der Recherche zur Besonderen Versorgung in den Fachgebieten, die verschiedenen Perspektiven, die Pretest-Ergebnisse, die Ergebnisse zu den aktuellen Projekten, der Struktur-, Prozess- und Ergebnisqualität sowie der Wirtschaftlichkeit werden präsentiert und diskutiert.
Instructional Quality and Learning Design of Massive Open Online Courses
Authors: Stracke Christian M. , Burgos Daniel , Tlili Ahmed 
Published: September 2022

AbstractThis chapter analyzes the instructional quality and learning design of different categories of online courses and their history, with a special focus on massive open online courses (MOOCs). Online courses have a long tradition that has gained public attention, broad interest, and huge numbers of participants thanks to the introduction of free MOOCs accessible online for all interested learners worldwide. In this chapter, we first define MOOCs, their characteristics, and history. Afterward, theoretical frameworks and practical instruments and tools based on scientific research are presented. From the beginning, the quality of MOOCs (and of online courses in general) has been debated. That led to discussions about the learning design and outcomes of MOOCs, which we introduce in the next section. Key research findings and practical validated instruments for designing and evaluating MOOCs (and online courses in general) are presented. Then following, the key benefits of MOOCs and the main arguments and scenarios for their usage are summarized. Based on our analysis of the research results, practices, and standards, a framework for categories and types of (massive open) online courses is proposed, called the typologies of online courses (TOC) framework. As part of the global community for open educational resources (OER) and in combination with the UNESCO recommendation on OER, MOOCs can play a significant role in achieving the SDG4 of the United Nations: inclusive and equitable quality education for all. This is true in particular during times of public lockdowns, such as during the ongoing COVID-19 pandemic.