bluuebunny/crossref_metadata_2025_split huggingface dataset:
Novel and very rare causative variants in the COL7A1 gene of Vietnamese patients with recessive dystrophic epidermolysis bullosa revealed by whole‐exome sequencing
Authors: Ma Thi Huyen Thuong , Luong Thi Lan Anh , Hoang Thu Lan , Nguyen Thi Thanh Hoa , Vu Thi Ha , Tran Van Khoa , Nguyen Duy Bac , Trieu Tien Sang , Nguyen Hai Ha , Nong Van Hai , Nguyen Dang Ton 
Published: July 2021

AbstractBackgroundDystrophic epidermolysis bullosa (DEB) is a rare inherited disorder characterized by skin fragility leading to trauma‐induced subepidermal blisters and healing with scarring. DEB is caused by mutations in COL7A1, the gene encoding for type VII collagen (COLVII). The DEB inheritance trait is divided into dominant dystrophic epidermolysis bullosa (DDEB) and recessive dystrophic epidermolysis bullosa (RDEB).MethodsWhole‐exome sequencing (WES) was performed for identifying mutations in six affected individuals of five Vietnamese families.ResultsThree novel variants in total of eight variants were found in five families. The first novel variant causing glycine substitution (c.8279G>A, p.G2760E), the remaining two novel variants resulted in splice site affecting (c.4518+2delT and c.5821‐2A>G). Functional analysis indicated that the splice site at c.4518+2delT resulted in a skipping of exon 43, leading to an in‐frame deletion of 12 amino acids.ConclusionOur finding expands the spectrum of COL7A1 mutations and reports altered splicing at c.4518+2delT during the processing of the pre‐mRNA. This study provides an additional scientific basis for diagnosis, genetic counseling, and prognosis purposes of EB patients.
Compact, broadband, wide angular stable circuit analog absorber at sub‐6 GHz for radar cross section reduction
Authors: Shukoor Mohammad Abdul , Dey Sukomal 
Published: July 2021

AbstractThis work presents a compact broadband wide angular stable polarization‐insensitive circuit analog absorber at sub 6 GHz. It shows almost 95% absorption from 2.26 to 5.76 GHz over the entire operating range. A transmission line equivalent circuit is modeled, and the response is validated. The design is obliquely stable up to 45° for both transverse electric and transverse magnetic incidences. The analysis of surface current distributions at two resonant frequencies confirming magnetic resonances' role in near‐unity absorption. It is compact with a periodicity of 0.1 λL and thickness of 0.08 λL. Finally, bistatic radar cross section reduction of the proposed absorber attached with an antenna is measured.
Interventional cardiac MRI using an add‐on parallel transmit MR system: In vivo experience in sheep
Authors: Godinez Felipe , Tomi‐Tricot Raphael , Delcey Marylène , Williams Steven E. , Mooiweer Ronald , Quesson Bruno , Razavi Reza , Hajnal Joseph V. , Malik Shaihan J. 
Published: July 2021

PurposeWe present in vivo testing of a parallel transmit system intended for interventional MR‐guided cardiac procedures.MethodsThe parallel transmit system was connected in‐line with a conventional 1.5 Tesla MRI system to transmit and receive on an 8‐coil array. The system used a current sensor for real‐time feedback to achieve real‐time current control by determining coupling and null modes. Experiments were conducted on 4 Charmoise sheep weighing 33.9‐45.0 kg with nitinol guidewires placed under X‐ray fluoroscopy in the atrium or ventricle of the heart via the femoral vein. Heating tests were done in vivo and post‐mortem with a high RF power imaging sequence using the coupling mode. Anatomical imaging was done using a combination of null modes optimized to produce a useable B1 field in the heart.ResultsAnatomical imaging produced cine images of the heart comparable in quality to imaging with the quad mode (all channels with the same amplitude and phase). Maximum observed temperature increases occurred when insulation was stripped from the wire tip. These were 4.1℃ and 0.4℃ for the coupling mode and null modes, respectively for the in vivo case; increasing to 6.0℃ and 1.3℃, respectively for the ex vivo case, because cooling from blood flow is removed. Heating < 0.1℃ was observed when insulation was not stripped from guidewire tips. In all tests, the parallel transmit system managed to reduce the temperature at the guidewire tip.ConclusionWe have demonstrated the first in vivo usage of an auxiliary parallel transmit system employing active feedback‐based current control for interventional MRI with a conventional MRI scanner.
A facile method for synthesizing polymeric nanofiber‐fragments
Authors: Banerjee Aihik , Jariwala Tanvi , Kim Sanggon , Tai Youyi , Chiang Sharon , Park Honghyun , Myung Nosang V. , Nam Jin 
Published: July 2021

AbstractElectrospinning is a versatile method for synthesizing nanofibrous structures from nearly all polymers, offering a solution for the industrial‐scale mass production of nanomaterials in a wide range of applications. However, the continuous non‐woven structure intrinsic to electrospun fibers limits their applications, where a smaller length scale is desired. Here, we present a novel method to synthesize polymeric nanofiber‐fragments based on colloid electrospinning of polymer and sacrificial silica nanoparticles, followed by mechanical fracturing with ultrasonication. The size and hydrophobicity of silica nanoparticles are optimized for their improved integration within the polymer matrix, and the controllability of nanofiber‐fragment length by the amount of silica nanoparticle loading, down to 2 µm in length for poly(vinylidene fluoride‐trifluoroethylene) nanofibers with an average fiber diameter of approximately 100 nm, is shown. The resultant nanofiber‐fragments are shown to maintain their material properties including piezoelectric coefficients and their enhanced injectability for drug delivery application is demonstrated with an animal model.
Transition care continuity promotes long‐term retention in adult care among young adults with sickle cell disease
Authors: Howell Kristen E. , Saulsberry‐Abate Anjelica C. , Mathias Joacy G. , Porter Jerlym S. , Hodges Jason R. , Ataga Kenneth I. , Anderson Sheila , Nolan Vikki , Hankins Jane S. 
Published: July 2021

AbstractBackground/objectivesCare continuity prevents increased health care utilization and mortality during transition from pediatric to adult care. Our program employs a co‐located care delivery model, in which pediatric provider involvement continues during young adulthood. We tested the hypothesis that individuals who participated in the co‐located model have greater retention in adult care compared to those who only received pediatric transition services.MethodsThis study consisted of 311 youth with SCD (51.4% male; 63.0% HbSS/HbSβ0‐thalassemia) who transferred to adult care from 2007 to 2017. Retention was defined as continuation with an adult provider for ≥12 or ≥24 months post‐pediatric care. Logistic regression estimated the association between co‐location status and retention at 12 and 24 months. Logistic regression and t‐tests were used to evaluate potential predictors of retention in adult care.ResultsIndividuals who participated in the co‐location model were 1.9 times more likely to remain in adult care 12 (95% CI: 1.01, 3.47) and 24 (95% CI: 1.01, 3.70) months post‐pediatric care compared to those who did not participate. Individuals with HbSS/HbSβ0‐thalassemia were 1.9 times more likely to be retained at 12 months compared to those with HbSC/HbSβ+‐thalassemia/HbS/HPFH (95% CI: 1.12, 3.09). For every clinic encounter in the last 2 years of pediatric care, the odds of being retained at least 24 months after initiating adult care increased 1.1 times (95% CI: 1.02, 1.13).ConclusionsContinuity of providers from pediatric to adult care may increase long‐term retention in adult care. Longitudinal monitoring of adult outcomes is critical to identifying the efficacy of transition services.
Microstructure‐Dependent Charge/Discharge Behaviors of Hollow Carbon Spheres and its Implication for Sodium Storage Mechanism on Hard Carbon Anodes
Authors: Chen Xiaoyang , Fang Youlong , Lu Haiyan , Li Hui , Feng Xiangming , Chen Weihua , Ai Xinping , Yang Hanxi , Cao Yuliang 
Published: July 2021

AbstractHard carbons are actively developed as a promising anode material for sodium ion batteries (SIBs). However, their sodium storage mechanism is poorly understood, leading to difficulties in design and development of high‐performance hard carbon anode materials. In this work, hollow carbon spheres (HCSs) with different shell thickness as a model material to investigate the correlation between the microstructural change and resulting Na+ storage behavior during charge/discharge cycles are designed and synthesized. Ex situ X‐ray diffraction and Raman evidences reveal that an interlayer spacing change of the graphitic nanodomains occurs in HCS electrode, leading to a shift of the reversible capacity from the high‐potential sloping (HPS) region to the low‐potential plateau (LPP) region. This unusual capacity shift suggests a microstructure‐dependent Na+ storage reaction on the HCS electrode and can be well explained by “adsorption‐intercalation” mechanism for these HCS materials. This work strengthens the understanding of the sodium storage behavior and provides a new perspective for the morphological and structural design of hard carbon anode materials for high‐performance SIBs.
Effect of Soy Waste/Cellulose on Mechanical, Water Sorption, and Biodegradation Properties of Thermoplastic Starch Composites
Authors: Behera Ajaya Kumar , Manna Suvendu , Das Nigamananda 
Published: July 2021

AbstractThermoplastic starch (TPS)/soy pulp (soy waste) composites (TSS) with varying compositions are successfully prepared in an internal mixer followed by compression molding. The mechanical, water absorption, and biodegradable properties of the developed composites are studied and compared with virgin TPS and cellulose reinforced TPS composites (TSC). Optimum tensile and flexural strength is observed for TSS and TSC composites with 10 wt.% soy waste and cellulose. Fourier Transform Infrared Spectroscopy (FT‐IR) and Scanning Electron Microscope (SEM) analyses indicated the interaction occurred between soy pulp/cellulose and TPS. Water absorptivity is found to increase from 6.2% to as high as 42.3% with the addition of 0 to 25 wt.% cellulose filler to the TPS matrix. Moreover, the soil burial degradation study shows higher degradability of the composites with a higher amount of soy pulp/cellulose. The developed composites show moderate mechanical properties with a hydrophobic and biodegradable nature. Therefore, there is a great possibility for these biocomposites to be utilized in applications such as disposable items, packaging materials, office cuboids, and transport sectors.
Attenuation of chloroquine and hydroxychloroquine on the invasive potential of bladder cancer through targeting matrix metalloproteinase 2 expression
Authors: Chou Kuang‐Yu , Chen Po‐Chun , Chang An‐Chen , Tsai Te‐Fu , Chen Hung‐En , Ho Chao‐Yen , Hwang Thomas I‐Sheng 
Published: July 2021

AbstractBladder cancer (BC), one of the most common urological neoplastic disorders in men, has an extremely low survival rate because of its tendency to metastasize. The anticancer drugs chloroquine (CQ) and hydroxy CQ (HCQ) might inhibit tumor progression and invasiveness. However, the mechanism by which CQ and HCQ influence BC is undetermined. In this study, CQ and HCQ treatments inhibited the migration and invasion of two BC cell types (5637 and T24) through expression modulation of matrix metalloproteinase‐2 (MMP‐2), which belongs to the matrix MMP family and is a key mediator of cancer progression. Moreover, additional data revealed that the migrative and invasive effects of BC cells treated with CQ or HCQ were abolished after treatment with rapamycin, which induces autophagy, demonstrating that CQ and HCQ functions in BC are based on autophagy inhibition. In conclusion, our research demonstrated that CQ and HCQ regulated cell motility in BC through MMP‐2 downregulation by targeting autophagy functions, providing a novel therapeutic strategy for BC treatment.
Speaking of Transition
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractWe are confronted with increasingly pressing questions about economic and social transition. Things have to change, but how? We believe that this change is concerned with three closely related challenges, which we call the triple transition—climate, energy, and circularity. These challenges combined result in a call for radical and major changes in the configuration of our economies and for the reconsideration and redesign of our systems. The fact remains that we live in organized societies and economies in which together we need to shape these transitions. Central to this book stands the premise that transition requires new forms of value creation materialized in the new generation of sustainable and circular business models. To make this possible the Business Model Template (BMT) was developed. The purpose of this chapter is to introduce you to the structure and style of this book and how the BMT is presented in the various chapters.
External Test
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractThe external test is the tenth and final building block of the first and second stage of the Business Model Template (BMT). You will test the viability of the business model concept you have developed so far. You can test your business model idea against all kinds of criteria. If you do not have much time, perform your check on the basis of the very concise set of seven questions we provide. But we also provide many other tools in this chapter. Please bear in mind that you must be well-prepared when looking for feedback. Look for people you know or hope will respond critically and constructively. Going through the External Test steps may mean that you need to partially adjust some elements of your business model before you move on. So be it. Remember that every version of your business model in this stage is a beta version and that feedback will help to make it better.
The Dream
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractLooking for your Dream provides a second tool to help you build your business model in this first stage of the Business Model Template (BMT). You have previously indicated which major social and/or ecological issues you want to solve. Now you will determine what the result of that solution could be. Look for a solution that goes beyond just tackling one issue. The Dream building block demands thinking big: it is a Big, Hairy, Audacious Goal (BHAG) on which you are going to work. How and why will your business model idea really make a difference? In order to not get lost in thinking about dreams, you could make use of various tools such as scenarios, metaphors or framing, all of which are addressed in this chapter. Putting all the steps in this chapter together, you will have dreamed up something big that you want to go for.
The Value Proposition
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractA business model is a description of how value creation is organized, underpinned by a value proposition. Such a proposition solves a problem or appeals to new, often yet undiscovered needs. Value creation has several facets. This building block offers a framework of five positions of value creation from which to choose and links it to strategies you can use. This in turn is then linked to the possibility of creating change with your business model-to-be. The more precisely you align the nature of those values and the change you would like to create, the better you will be able to design an appropriate (organizational) logic at a later stage. Formulating a clear and compelling value proposition is crucial in the development of a business model. It gives direction to the strategy, to the stakeholders with whom you could take these steps, and to what impact you expect to realize.
Parties Involved
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractThe central question in this building block of the Business Model Template (BMT) is identifying what stakeholders and other parties are pivotal for your success. Here you make an overview of them, not only determining who they are but also what interests they have in relation to your business model. Numerous tools have been developed to identify stakeholders. We will provide you with various tools to make a balanced identification. Defining the interests, influence and interrelationships between the stakeholders in your network helps to determine the connections that must be reinforced or made in order for your business model to function. In addition it helps in understanding who can play a crucial role in helping to create a fit between your business model and its environment. Finally, reflection on the key stakeholders and their roles is vital because of the input for the choice of strategy underpinning your business model.
Core Activities
Authors: Jonker Jan , Faber Niels 
Published: July 2021

AbstractIn this chapter, the central question is which core activities you will undertake in order to achieve your goal, organizing your value proposition with success. Because you are working towards a specific goal, and with selected strategies, it is useful to state which (core) activities are necessary to realize your sustainable business model. The idea of a core activity is that a specific part of the organizational activities can be seen as the speciality of a company, a network, or a community: it tells what they are really good at. Core activities should contribute to operationalizing the chosen strategy, thus contributing to the realization of the overall goal, coherent with the value proposition. We offer a core activities framework based on the conventional sustainability trio: reduce, reuse, recycle. This has evolved over the years into a whole family—commonly referred to as the RE-strategies and presented here as the 13 REs.
„Der Andere“ und zugleich „der Gleiche“ sein: Vom Umgang männlicher Kinderbetreuer mit Identitätsdissonanz
Authors: Nentwich Julia C. , Tennhoff Wiebke 
Published: July 2021

ZusammenfassungMänner in der Kita sehen sich mit widersprüchlichen „Anrufungen“ konfrontiert. Zum einen müssen sie Erwartungen an die Männlichkeit ihrer Person gerecht werden, zum anderen aber auch als „Gleiche“ handeln, wollen sie das weiblich konnotierte Ideal der guten Kinderbetreuerin erfüllen. Mit der entstehenden Identitätsdissonanz umgehen zu können wird zu einer grundlegenden Anforderung an Männer in einem Frauenberuf. Mit einer diskurspsychologischen Analyse arbeiten wir insgesamt sechs diskursive Praktiken heraus, mittels derer es den zehn von uns interviewten männlichen Kinderbetreuern gelingt, im Interview trotz erlebter Identitätsdissonanz verschiedene legitime Subjektpositionen zu etablieren.
(Un)doing gender empirisch: Konzeptionelle, methodische und praktische Schlussfolgerungen
Authors: Nentwich Julia C. , Vogt Franziska 
Published: July 2021

ZusammenfassungIm Forschungsprojekt „(Un)doing gender in der Kita“ haben wir vielfältige theoretische Konzeptionen von doing und undoing gender empirisch untersucht. Im folgenden Kapitel stellen wir die hierfür entwickelte Methodologie des Explizierens, Reflektierens und Kontrastierens sowie den Zugang einer multimethodischen und mehrperspektivischen Ethnografie dar. Die so erzielten Ergebnisse sind auch für die Praxis relevant. Führung und Zusammenarbeit, die Inklusion von Männern in das Team wie auch eine geschlechtergerechte Pädagogik profitieren von im Rahmen von Organisationsentwicklungsprozessen gemachten Reflexionen.
Exact-WKB, complete resurgent structure, and mixed anomaly in quantum mechanics on S1
Authors: Sueishi Naohisa , Kamata Syo , Misumi Tatsuhiro , Ünsal Mithat 
Published: July 2021

Abstract
We investigate the exact-WKB analysis for quantum mechanics in a periodic potential, with N minima on S1. We describe the Stokes graphs of a general potential problem as a network of Airy-type or degenerate Weber-type building blocks, and provide a dictionary between the two. The two formulations are equivalent, but with their own pros and cons. Exact-WKB produces the quantization condition consistent with the known conjectures and mixed anomaly. The quantization condition for the case of N-minima on the circle factorizes over the Hilbert sub-spaces labeled by discrete theta angle (or Bloch momenta), and is consistent with ’t Hooft anomaly for even N and global inconsistency for odd N. By using Delabaere-Dillinger-Pham formula, we prove that the resurgent structure is closed in these Hilbert subspaces, built on discrete theta vacua, and by a transformation, this implies that fixed topological sectors (columns of resurgence triangle) are also closed under resurgence.
K → μ+μ− as a clean probe of short-distance physics
Authors: Dery Avital , Ghosh Mitrajyoti , Grossman Yuval , Schacht Stefan 
Published: July 2021

AbstractTheK→μ+μ−decay is often considered to be uninformative of fundamental theory parameters since the decay is polluted by long-distance hadronic effects. We demonstrate that, using very mild assumptions and utilizing time-dependent interference effects, ℬ(KS→μ+μ−)ℓ=0can be experimentally determined without the need to separate theℓ= 0 andℓ= 1 final states. This quantity is very clean theoretically and can be used to test the Standard Model. In particular, it can be used to extract the CKM matrix element combination$$ \mid {V}_{ts}{V}_{td}\sin \left(\beta +{\beta}_s\right)\mid \approx \mid {A}^2{\lambda}^5\overline{\eta}\mid $$∣VtsVtdsinβ+βs∣≈∣A2λ5η¯∣with hadronic uncertainties below 1%.
History and evolution of the afroalpine flora: in the footsteps of Olov Hedberg
Authors: Brochmann Christian , Gizaw Abel , Chala Desalegn , Kandziora Martha , Eilu Gerald , Popp Magnus , Pirie Michael D. , Gehrke Berit 
Published: July 2021

AbstractThe monumental work of Olov Hedberg provided deep insights into the spectacular and fragmented tropical alpine flora of the African sky islands. Here we review recent molecular and niche modelling studies and re-examine Hedberg’s hypotheses and conclusions. Colonisation started when mountain uplift established the harsh diurnal climate with nightly frosts, accelerated throughout the last 5 Myr (Plio-Pleistocene), and resulted in a flora rich in local endemics. Recruitment was dominated by long-distance dispersals (LDDs) from seasonally cold, remote areas, mainly in Eurasia. Colonisation was only rarely followed by substantial diversification. Instead, most of the larger genera and even species colonised the afroalpine habitat multiple times independently. Conspicuous parallel evolution occurred among mountains, e.g., of gigantism inLobeliaandDendrosenecioand dwarf shrubs inAlchemilla. Although the alpine habitat was ~ 8 times larger and the treeline was ~ 1000 m lower than today during the Last Glacial Maximum, genetic data suggest that the flora was shaped by strong intermountain isolation interrupted by rare LDDs rather than ecological connectivity. The new evidence points to a much younger and more dynamic island scenario than envisioned by Hedberg: the afroalpine flora is unsaturated and fragile, it was repeatedly disrupted by the Pleistocene climate oscillations, and it harbours taxonomic and genetic diversity that is unique but severely depauperated by frequent bottlenecks and cycles of colonisation, extinction, and recolonisation. The level of intrapopulation genetic variation is alarmingly low, and many afroalpine species may be vulnerable to extinction because of climate warming and increasing human impact.