bluuebunny/crossref_metadata_2025 huggingface dataset:
From neural stem cells to glioblastoma: A natural history of GBM recapitulated in vitro
Authors: Almengló Cristina , Caamaño Pilar , Fraga Máximo , Devesa Jesús , Costoya José A. , Arce Víctor M.
Published: May 2021

Due to its aggressive and invasive nature glioblastoma (GBM), the most common and aggressive primary brain tumour in adults, remains almost invariably lethal. Significant advances in the last several years have elucidated much of the molecular and genetic complexities of GBM. However, GBM exhibits a vast genetic variation and a wide diversity of phenotypes that have complicated the development of effective therapeutic strategies. This complex pathogenesis makes necessary the development of experimental models that could be used to further understand the disease, and also to provide a more realistic testing ground for potential therapies. In this report, we describe the process of transformation of primary mouse embryo astrocytes into immortalized cultures with neural stem cell characteristics, that are able to generate GBM when injected into the brain of C57BL/6 mice, or heterotopic tumours when injected IV. Overall, our results show that oncogenic transformation is the fate of NSC if cultured for long periods in vitro. In addition, as no additional hit is necessary to induce the oncogenic transformation, our model may be used to investigate the pathogenesis of gliomagenesis and to test the effectiveness of different drugs throughout the natural history of GBM.
Concentration‐dependent duality of bFGF in regulation of barrier properties of human brain endothelial cells
Authors: Kriaučiūnaitė Karolina , Pociūtė Agnė , Kaušylė Aida , Pajarskienė Justina , Verkhratsky Alexei , Pivoriūnas Augustas
Published: May 2021

Multiple paracrine factors regulate the barrier properties of human brain capillary endothelial cells (BCECs). Understanding the precise mode of action of these factors remains a challenging task, because of the limited availability of functionally competent BCECs and the use of serum‐containing medium. In the present study, we employed a defined protocol for producing BCECs from human inducible pluripotent stem cells. We found that autocrine secretion of basic fibroblast growth factor (bFGF) is necessary for the establishment a tight BCECs barrier, as revealed by measurements of transendothelial electric resistance (TEER). In contrast, addition of exogenous bFGF in concentrations higher than 4 ng/ml inhibited TEER in a concentration‐dependent manner. Exogenous bFGF did not significantly affect expression and distribution of tight junction proteins claudin‐5, occludin and zonula occludens (ZO)‐1. Treatment with FGF receptor blocker PD173074 (15 µM) suppressed inhibitory effects of bFGF and induced nuclear translocation of protein ZO‐1. Inhibition of phosphoinositide 3‐Kinase (PI‐3K) with LY294002 (25 µM) significantly potentiated an inhibitory effect of bFGF on TEER indicating that PI‐3K signalling pathway counteracts bFGF modulation of TEER. In conclusion, we show that autocrine bFGF secretion is necessary for the proper barrier function of BCECs, whereas exogenous bFGF in higher doses suppresses barrier resistance. Our findings demonstrate a dual role for bFGF in the regulation of BCEC barrier function.
Mycobacterium tuberculosis effector PPE36 attenuates host cytokine storm damage via inhibiting macrophage M1 polarization
Authors: Gong Zhen , Han Shuang , Liang Tian , Zhang Hongyang , Sun Qingyu , Pan Huimin , Wang Haolin , Yang Jiao , Cheng Liting , Lv Xi , Yue Qijia , Fan Lin , Xie Jianping
Published: May 2021

Tuberculosis caused by Mycobacterium tuberculosis remains a serious global public health threat. Macrophage polarization is crucial for the innate immunity against M. tuberculosis. However, how M. tuberculosis interferes with macrophage polarization is elusive. We demonstrated here that M. tuberculosis PPE36 (Rv2108) blocked macrophage M1 polarization, preventing the cytokine storm, and alleviating inflammatory damage to mouse immune organs. PPE36 inhibited the polarization of THP‐1 cell differentiation to M1 macrophages, reduced mitochondrial dehydrogenase activity, inhibited the expression of CD16, and repressed the expression of pro‐inflammatory cytokines IL‐6 and TNF‐α, as well as chemokines CXCL9, CXCL10, CCL3, and CCL5. Intriguingly, in the mouse infection model, PPE36 significantly alleviated the inflammatory damage of immune organs caused by a cytokine storm. Furthermore, we found that PPE36 inhibited the polarization of macrophages into mature M1 macrophages by suppressing the ERK signaling. The study provided novel insights into the function and mechanism of action of M. tuberculosis effector PPE36 both at the cellular and animal level.
Enhancement of nitrate removal under limited organic carbon with hydrogen‐driven autotrophic denitrification in low‐cost electrode bio‐electrochemical reactors
Authors: Peungtim Prarunchaya , Meesungnoen Orapan , Mahachai Palisa , Subsoontorn Pakpoom , Do Thu Nga , Nakaruk Auppatham , Khanitchaidecha Wilawan
Published: May 2021

BACKGROUND

Nitrate‐contaminated water is a concerning environmental and health problem in agricultural areas. Due to its low organic carbon content, such water cannot be purified using a conventional bioreactor with heterotrophic denitrification. Low‐cost bio‐electrochemical reactors were established with the aim of enhancing denitrification performance by cooperation of heterotrophic and hydrogenotrophic denitrification.

RESULTS

At the lowest C/N ratio of 1.0, the bioreactor reached only 67.4% for NO3‐N removal and 47.5% for total N removal (total N removal rate of ca 1.2 mg L−1 h−1), whereas the Cu reactor (bio‐electrochemical reactor using copper wire as cathode) achieved the best efficiencies of 73.7% and 53.8% for NO3‐N and total N removal (total N removal rate of ca 1.3 mg L−1 h−1), and followed by the SS reactor (bio‐electrochemical reactor using stainless steel wire as cathode). On the other hand, the greatest total organic carbon (TOC) removal efficiency was observed for the bioreactor and followed by the SS reactor and the Cu reactor. The low TOC consumption of 1.2–1.4 mg‐TOC (mg‐N)−1 in the Cu reactor and the SS reactor indicated the enhanced denitrification from coexisting hydrogenotrophic denitrification. In addition, the performance of hydrogenotrophic denitrification was improved by increasing the applied current from 10 to 30 mA.

CONCLUSIONS

The abundance of autotrophic denitrifying bacteria (identified as hydrogenotrophs) was higher than that of heterotrophic denitrifying bacteria in both the Cu reactor and the SS reactor. Archromobacter and Flavobacterium were the most dominant genera in the biofilm of the Cu reactor cathode and in the suspended sludge, respectively. Hydrogenophaga were detected in both biomass samples, but were absent from the biomass samples of the conventional bioreactor. © 2021 Society of Chemical Industry (SCI).
Topographic characterization of (Zr, Mn) co‐doped bismuth ferrite thin film surfaces
Authors: Ţălu Ştefan , Priya Arumugasamy Sathiya , Geetha Deivasigamani
Published: May 2021

The present work focuses on the investigations on the optical and morphological characteristics of (Zr, Mn) co‐doped Bismuth ferrite thin films. Undoped Bismuth ferrite (BiFeO3) and (Zr, Mn) co‐doped Bismuth ferrite (ZMBFO) thin films were spin‐coated on Pt/TiO2/Ti/SiO2/Si substrate. X‐ray diffraction (XRD) was utilized to confirm the rhombohedral structure and the Scherrer formula was used to calculate the crystallite size of the films. The optical properties were explored using UV–visible spectroscopy and the bandgap energies were calculated using a Tauc plot. The surface morphology of the films was explored with the help of atomic force microscopy (AFM) and the three‐dimensional (3‐D) surface features were determined using stereometric analyses. The variation of 3‐D surface parameters was identified to be the consequence of doping.
Temporary cement residues affect the bond strength and dentin penetration of self‐adhesive resin cement in fiberglass post cementation
Authors: Zaniboni Joissi Ferrari , Silva Aryvelto Miranda , Fernández Eduardo , de Melo Alencar Cristiane , Morais Jéssika Mayhara Pereira , de Campos Edson Alves , Kuga Milton Carlos
Published: May 2021

This study evaluated the persistence of eugenol‐containing (PR, Provy) or eugenol‐free (RT, Relyx Temp, and TB, Temp Bond) temporary cement residues and its effects on push‐out bond strength and dentinal penetration of the self‐adhesive resin cement (Relyx U200). Eighty human roots were endodontically treated and post space prepared. Fourty specimens were distributed in four groups (n = 10): Control (CO), without any clinical procedure, PR, RT, and TB, where a metallic post was cemented with one of the temporary cements. After metallic post removal, hemi sections post space were submitted to SEM analysis. Another specimens were distributed in similar groups, but fiberglass post was cemented using Relyx U200 cement and submitted to push‐out bond strength and dentinal penetration cement analysis, in post space thirds. SEM analysis were evaluated by Kruskal–Wallis and Dunn tests, while bond strength and dentinal penetration were evaluated by ANOVA One‐Way and Tukey test (p < .05). Only in apical thirds, PR, RT, and TB showed higher persistence of residues than CO. In bond strength, PR, RT, and TB showed lower values than CO, in all post space thirds (p < .05). But to dentinal penetration, PR, RT, and TB presented lower values than CO only in apical thirds (p < .05). There is the greatest persistence of temporary cement residues only in apical post space third, providing less dentinal penetration of the self‐adhesive resinous cement. However, the previous use of temporary cements has negative effects on the bond strength cementation system, regardless of the third evaluated.
Allogenic hematopoietic stem cell transplantation in two siblings with adult metachromatic leukodystrophy and a systematic literature review
Authors: Videbæk Cecilie , Stokholm Jette , Sengeløv Henrik , Fjeldborg Lone U. , Larsen Vibeke Andrée , Krarup Christian , Nielsen Jørgen E. , Grønborg Sabine
Published: May 2021

Two siblings were diagnosed with adult metachromatic leukodystrophy (MLD) and treated with hematopoietic stem cell transplantation (HSCT). While the older sibling was symptomatic at the time of diagnosis, her younger brother was diagnosed and transplanted at the presymptomatic state. We describe patients' clinical, biochemical, and genetic features, as well as neuropsychological and neurophysiological test results, and brain magnetic resonance imaging from pretransplantation and posttransplantation assessments. Both patients converted to complete donor chimerism and arylsulfatase A levels normalized 3 months posttransplantation. Twelve months posttransplantation, neurological and neuropsychological assessment for both patients showed stabilization, and they remained stable for the 38 months long observation period. To assess the effect of HSCT used as treatment for the rare, adult MLD subtype on survival and stabilization, we performed a systematic literature review and included 7 studies with a total of 26 cases. Of these 26 cases, 6 patients died of HSCT‐related complications and 2 patients had graft rejection. Of the remaining 18 patients, 2 patients improved after HSCT, 13 patients stabilized, and 3 patients progressed, suggesting that HSCT potentially benefits adult MLD patients. Larger studies focusing on this subtype are needed and recommendations on criteria for HSCT in adult MLD need to be evolved.
Analytical modeling and optimization of new Ku‐band tunable square waveguide iris‐post polarizer
Authors: Piltyay Stepan , Bulashenko Andrew , Sushko Oleksandr , Bulashenko Oleksandr , Demchenko Ivan
Published: May 2021

A novel design of the polarizer based on a square waveguide with four posts and four irises for Ku‐band 10.7–12.8 GHz is proposed. The main advantage of the developed polarizer design is the option to tune its polarization characteristics by variation of the posts height. The equivalent circuit of the polarizer has been obtained using scattering and transfer matrices technique. Using this model, the polarization and matching characteristics of the suggested polarizer were optimized simultaneously using the trust region method. This method is reliable and robust since it provides good convergence properties. The novelty of this study lies in the use of a new waveguide polarizer structure based on a combination of irises and posts. Posts enable fine‐tuning of the polarizer performance, while irises promote wide operating frequency band. The following simulated characteristics of the optimal polarizer design were obtained: VSWR for the main modes of horizontal and vertical polarization is less than 1.47, the differential phase shift is within 90° ± 2.6°, the axial is less than 0.46 dB, and the XPD is higher than 32 dB in the operating frequency range. A developed polarizer can be applied in antenna feeds for satellite communication and radar systems.
Predictive relationships between winter climate and hot season temperatures over India
Authors: Dubey Aditya Kumar , Kumar Pankaj
Published: May 2021

Climate change has impacted the nature and the severity of extreme temperatures over India in the recent past. In this study, the characterization in nature and change of hot season (March–May) temperatures across India's various geographical regions have been analysed over the last 69 years. Using canonical correlation analysis (CCA), we elucidate the relationships between various meteorological parameters from the preceding Indian winter (December–February) and the following hot season. A combination of detrended and raw data for CCA has revealed the asynchronous winter parameter's effects on hot season temperatures. The raw data explained the evolution of the relationship between the variables in the warming climate. In contrast, detrended data explains the predictive relationship between hot season temperature and other predictive variables which are not influenced by the trend in the data. The evolving nature of the global and Indian ocean as well as, El‐Niño Southern Oscillation, zonal circulation, and pressure systems, are evident in effecting Indian hot season temperatures. The post‐1980s warming is also tangible from the increasing strength of El Niño in the last few decades. The cross‐validated and mean square skill scores are moderate except for a few regions having an average correlation of ~.5 or more for the study periods. The minimum temperature has shown better skill than the maximum temperature, and the southern peninsular region has shown more predictability than the northern regions. Compared to raw data, detrended has a lesser prediction skill. The present study will help to predict the seasonal temperature along with the associated relationship and the warming effect post‐1980s.
Phase Behavior of Non‐Ionic Surfactant‐Medium Chain Triglyceride‐Water Microemulsion Systems
Authors: Callender Shannon P. , Wettig Shawn D.
Published: May 2021

Microemulsion systems have garnered tremendous interest in the pharmaceutical sector for a variety of drug delivery applications. Non‐ionic surfactants are often the preferred surfactant class given their uncharged nature, enhanced oral safety profile, and generally regarded as safe status as compared to other surfactant classes (Myers, Surfactant science and technology, 2005, p. 29), (Malmsten, Handbook of microemulsion science and technology, 1999, p. 755), (Grove & Mullertz, Chapter 5‐liquid self‐microemulsifying drug delivery systems, 2007), (Liu et al., Water‐insoluble drug formulation, 2008), (Hauss, Advanced Drug Delivery Reviews, 2007, 59, pp. 667–676), (Balazs, Solubility, delivery and ADME problems of drugs and drug‐candidates, 2011, p. 68). In this work, the phase behavior and microemulsion formation potential of four commonly used non‐ionic surfactants, PEG‐40 hydrogenated castor oil, Poloxamer 188, Polysorbate 80, and d‐α‐tocopherol polyethylene glycol succinate were studied via ternary phase diagram (TPD) mapping using a medium chain triglyceride, Miglyol 812. Results indicated notable differences in phase behavior despite similarities in hydrophilic–lipophilic balance value (13–15). All surfactants produced Winsor Type I, oil‐in‐water microemulsions at water concentrations above 40% wt/wt. Winsor Type II water‐in‐oil microemulsions were difficult to obtain even at high oil concentrations of ≥70% wt/wt. Winsor III microemulsions, though rare, were generally obtained in the middle regions of the TPD between 10% and 30% wt/wt water while Winsor IV microemulsions dominated at high surfactant concentrations of ≥45% wt/wt. Opaque emulsion areas were particularly notable in wax state surfactants. Polysorbate 80 and PEG‐40 hydrogenated castor oil demonstrated a high degree of synergism as well as the largest oil‐in‐water (o/w) and water‐in‐oil (w/o) microemulsion formation potential rendering them suitable for a number of enteral and parenteral applications.
The learning curve for uniportal video‐assisted thoracoscopic anatomical segmentectomy
Authors: Li Shenghui , Wu Junqi , Wan Ziwei , Chen Yingyao , She Yunlang , Xie Dong , Hu Xuefei , Zhao Deping , Chen Chang
Published: May 2021

Objectives

The aim of this study is to evaluate the time course and caseload required to achieve proficiency by plotting the learning curve of uniportal thoracoscopic segmentectomy.

Methods

We retrospectively analyzed the first 238 and 159 cases of uniportal thoracoscopic segmentectomy performed by two surgeons (A and B). The learning curves were assessed using cumulative sum analysis. Perioperative outcomes were evaluated as the learning curve developed. Two subtypes of this surgical approach, simple and complex segmentectomy, were separately analyzed.

Results

Based on the learning curve, the inflection points occurred at 64 and 90 cases for surgeon A, 71 and 100 cases for surgeon B. Significantly longer operative time (p = .013), length of stay (p = .002), and drainage duration (p = .039) were observed between phase I and phase II compared to phase III for surgeon A. Operative times (p = .001) were significantly reduced for surgeon B. Furthermore, 26‐28 and 52‐56 cases were necessary to master the simple and complex segmentectomy, respectively.

Conclusions

A total 64–71 cases were required to master uniportal thoracoscopic segmentectomy and 90–100 cases were necessary to achieve proficiency.
Auditory N2 Correlates of Treatment Response in Posttraumatic Stress Disorder
Authors: Tillman Gail D. , Motes Michael A. , Bass Christina M. , Morris Elizabeth Ellen , Jones Penelope , Kozel F. Andrew , Hart John , Kraut Michael A.
Published: May 2021

Emotional processing and cognitive control are implicated as being dysfunctional in posttraumatic stress disorder (PTSD) and targeted in cognitive processing therapy (CPT), a trauma‐focused treatment for PTSD. The N2 event‐related potential has been interpreted in the context of emotional processing and cognitive control. In this analysis of secondary outcome measures from a randomized controlled trial, we investigated the latency and amplitude changes of the N2 in responses to task‐relevant target tones and task‐irrelevant distractor sounds (e.g., a trauma‐related gunshot and a trauma‐unrelated lion's roar) and the associations between these responses and PTSD symptom changes. United States military veterans (N = 60) diagnosed with combat‐related PTSD were randomized to either active or sham repetitive transcranial magnetic stimulation (rTMS) and received a CPT intervention that included a written trauma account element (CPT+A). Participants were tested before and 6 months after protocol completion. Reduction in N2 amplitude to the gunshot stimulus was correlated with reductions in reexperiencing, |r| = .445, and hyperarousal measures, |r| = .364. In addition, in both groups, the latency of the N2 event‐related potential to the distractors became longer with treatment and the N2 latency to the task‐relevant stimulus became shorter, ηp2 = .064, both of which are consistent with improved cognitive control. There were no between‐group differences in N2 amplitude and latency. Normalized N2 latencies, reduced N2 amplitude to threatening distractors, and the correlation between N2 amplitude reduction and PTSD symptom reduction reflect improved cognitive control, consistent with the CPT+A objective of addressing patients’ abilities to respond more appropriately to trauma triggers.
Oxidation of aniso‐paraffinic alcohol‐to‐jet fuel and n‐heptane mixture: An experimental and modeling study
Authors: Guzman Juan , Kukkadapu Goutham , Brezinsky Kenneth , Westbrook Charles K.
Published: May 2021

Oxidation of alcohol to jet (ATJ)/n‐heptane blends was studied over a wide range of test conditions, using single pulse shock tubes. Test conditions were designed to study the effect of pressure (4 and 50 bar), and fuel loading (∼100 to 1400 ppm) on the oxidation of the blends across a wide range of temperatures (800–1300 K). These effects were observed by measuring concentrations of intermediate species formed using in‐line gas chromatography (GC) and GCxGC TOF‐MS. Results showed that increasing the initial fuel load does not have a significant impact on the results. However, increasing the pressure shifts the mixture reactivity to a lower temperature by about 150 K and causes the fuel to be oxidized instead of decomposing pyrolytically as at lower pressures. Additional experiments for pure ATJ and puren‐heptane were performed at conditions matching the ATJ/n‐heptane 50 bar experiments to analyze the differences between the pure and mixed fuels. Speciation measurements were compared against predictions from a detailed kinetic model. The ability of the kinetic model to capture the effects of varying experimental test conditions on the evolution of intermediate species is discussed, and kinetic analyses have been conducted to identify the important reaction pathways.
SITES AquaNet: An open infrastructure for mesocosm experiments with high frequency sensor monitoring across lakes
Authors: Urrutia‐Cordero P. , Langvall O. , Blomkvist P. , Angeler D.G. , Bertilsson S. , Colom Montero W. , Eklöv P. , Aagaard Jakobsen N. , Klemedtsson L. , Laudon H. , Liljebladh B. , Lundgren M. , Parkefelt L. , Kelpsiene E. , Pierson D. , Rankinen J. , Striebel M. , Tranvik L. J. , Weslien P. , Hillebrand H. , Langenheder S.
Published: May 2021

For aquatic scientists mesocosm experiments are important tools for hypothesis testing as they offer a compromise between experimental control and realism. Here we present a new mesocosm infrastructure—SITES AquaNET—located in five lakes connected to field stations in Sweden that cover a ~760 km latitudinal gradient. SITES AquaNet overcomes major hindrances in aquatic experimental research through: (i) openness to the scientific community, (ii) the potential to implement coordinated experiments across sites and time, and (iii) high‐frequency measurements (temperature, photosynthetic photon flux density, turbidity and dissolved oxygen, chlorophyll a and phycocyanin concentrations) with an autonomous sensor system. Moreover, the infrastructure provides operational guidance and sensor expertise from technical staff, and connections to a multi‐layered monitoring programme (“SITES Water”) for each lake. This enables ecological observations from whole lake ecosystems to be compared with experimental studies aiming at disentangling major drivers and mechanisms underlying observed changes. Here we describe the technical properties of the infrastructure along with possibilities for experimental manipulations to tackle pressing issues in aquatic ecology and global change science. As a proof of concept, we also present a first mesocosm experiment across all five field sites with a cross‐factorial design to evaluate responses of the sensor measurements to press/bottom‐up (constant light reduction) and pulse/top‐down (temporary fish predation) disturbances. This demonstrates the suitability of the infrastructure and autonomous sensor system to host modularized experiments and exemplifies the power and advantages of the approach to integrate a network of mecsocosm facilities with manageable costs across large geographic areas.
Penta‐Hydroxy Flavones‐Based Photoinitiating Systems for Free Radical, Cationic, and Thiol‐Ene Polymerization upon Exposure to Mild Blue LEDs
Authors: Zhu Di , Peng Xiaotong , Xiao Pu
Published: May 2021

Natural flavone‐derivatives (i.e., morin and quercetin) are investigated as visible light‐sensitive photoinitiators. The photochemical mechanisms of the radical generation from flavone‐derivative‐based photoinitiating systems (PISs) upon exposure to mild blue light‐emitting diodes are studied. The impact of substituent site of functional groups on the photoinitiation ability is determined. The quercetin‐based PISs exhibit more reactivity than the morin‐based systems on free radical photopolymerization. Moreover, the investigated flavone‐based PISs present their stabilities at room temperature, indicating that resin containing these systems barely require special storage requirements. Thiol‐ene polymerization initiated by the flavone‐derivatives‐based PISs is also investigated.
Highly Tough and Thermally Stable Polylactide Blends Compatibilized with Glycidyl Methacrylate‐Grafted Polypropylene
Authors: Lee Ji‐Su , Hwang Gyu‐Hyun , Kwon Young Seung , Jeong Young Gyu
Published: May 2021

To attain eco‐friendly and sustainable polylactide (PLA) materials possessing highly enhanced toughness, thermal stability, and processability without significant loss in elastic modulus, for the first time, PLA‐dominant blends with 1–30 wt% glycidyl methacrylate‐grafted polypropylene (PPGMA) loadings are fabricated via an efficient masterbatch melt‐compounding process. For the purpose, PPGMA is fabricated via in situ grafting reaction of PP with GMA and styrene. The scanning electron microscope images reveal that PLA/PPGMA blends do not show recognizable phase‐separated domains, unlike immiscible PLA/PP blends. The Fourier‐transform infrared spectroscopic and melt‐rheological analyses support the presence of specific interactions between PLA and PPGMA as well as the compatibilizing effect of PPGMA‐g‐PLA formed during the melt‐compounding. The thermal analyses demonstrate that PPGMA component accelerates the crystallization of PLA in the blends and that the thermal decomposition temperatures of PLA/PPGMA blends are higher than those of neat PLA and PPGMA components. The dynamic mechanical analysis shows that a maximum storage modulus is attained for PLA‐dominant blend with 30 wt% PPGMA. Noticeably, the impact strength (≈305.6 J m−1) of PLA‐dominant blend with only 5 wt% PPGMA loading is almost three times higher than that (≈111.6 J m−1) of neat PLA and it is very comparable to the value (≈316.9 J m−1) of neat PP.
Physicochemical Properties of Injectable Hyaluronic Acid: Skin Quality Boosters
Authors: Kleine‐Börger Linda , Kalies Alexander , Meyer Robert SA , Kerscher Martina
Published: May 2021

In addition to clinical research on the efficacy and compatibility of injectable hyaluronic acid (HA) based fillers, ample in vitro research on the physicochemical properties of these materials is published. Different HA‐based product ranges are also available as skin quality boosters (SQBs) to improve skin quality. The aim of this study is to investigate the physicochemical properties of specific HAs for improving skin quality, as, to the best of the authors' knowledge, no data is published on this topic to date. This paper may provide a better understanding of clinical performance and differentiation between these SQBs. Five HA injectable hydrogels (Belotero Revive, Juvederm Volite, Restylane Skinbooster Vital, Viscoderm Hydrobooster, and Profilho) from the SQB product ranges of different companies and manufacturing technologies are investigated for their extrusion force, swelling degree, rheological performance, and cohesivity. There are significant differences in extrusion force, swelling degree, rheological performance, and cohesivity between the assessed SQBs. HA concentration (mg mL−1) exhibits statistically significant positive correlations with extrusion force, swelling degree, tan delta, and cohesivity. This study provides a physicochemical characterization of different SQBs and information to improve understanding of this type of product.
β‐Pinene‐Derived Polyesteramides and Their Blends: Advances in Their Upscaling, Processing, and Characterization
Authors: Kleybolte Magdalena Maria , Winnacker Malte
Published: May 2021

Terpene‐based polyesteramides (PEAs) are sustainable and have a variety of favorable properties, making them suitable for a wide range of applications and for contribution to a much more sustainable polymer industry. This work focuses on the synthesis of the lactam fromβ‐pinene and its copolymerization withε‐caprolactone. An important step in synthesizingβ‐pinene lactam is the oxidation of β‐pinene to nopinone. To make the established oxidative cleavage more sustainable and efficient, the required amounts of Al2O3and KMnO4are significantly reduced by using H2SO4as a catalyst. For the Beckmann rearrangement various catalysts and co‐reagents are screened. Among these, the reaction with tosyl chloride is found the most favorable. Subsequently, the chain lengths of theβ‐pinene‐based PEAs are remarkably increased from 6000 g mol−1to more than 25 100 g mol−1by fine‐tuning reaction time, temperature, and decreasing catalyst and initiator concentrations. Also, different catalysts for polymerization are tested. The resulting material shows melting temperatures of ≈55 °C and decomposition temperatures of 354 °C or higher. Processing via melt pressing or casting turned out to be quite difficult due to the polymer's brittleness. Furthermore, regarding biomedical applications, blends of PEA with polyethylene glycol were successfully prepared, yielding a more hydrophilic material.
Valuation and pricing of software licenses to support supplier–buyer negotiations: A case study in the automotive industry
Authors: Bodendorf Frank , Lutz Manuel , Franke Jörg
Published: May 2021

Licensed standard software can be replicated millions of times at near zero cost for the supplier and poses immense challenges for purchasing and cost managers. A model is introduced to evaluate the appropriateness of software prices and thus to support the negotiation and decision process regarding suppliers. The model determines the “value to customer” of a software product using conjoint analysis and converts that value into a theoretical license price. In a case study coming from a large Original Equipment Manufacturer (OEM) it is shown that the developed conjoint model can successfully be used in practice and suggests realistic purchase prices.
X‐Linked Parkinsonism: Phenotypic and Genetic Heterogeneity
Authors: Di Lazzaro Giulia , Magrinelli Francesca , Estevez‐Fraga Carlos , Valente Enza M. , Pisani Antonio , Bhatia Kailash P.
Published: May 2021

X‐linked parkinsonism encompasses rare heterogeneous disorders mainly inherited as a recessive trait, therefore being more prevalent in males. Recent developments have revealed a complex underlying panorama, including a spectrum of disorders in which parkinsonism is variably associated with additional neurological and non‐neurological signs. In particular, a childhood‐onset encephalopathy with epilepsy and/or cognitive disability is the most common feature. Their genetic basis is also heterogeneous, with many causative genes and different mutation types ranging from “classical” coding variants to intronic repeat expansions. In this review, we provide an updated overview of the phenotypic and genetic spectrum of the most relevant X‐linked parkinsonian syndromes, namely X‐linked dystonia‐parkinsonism (XDP, Lubag disease), fragile X‐associated tremor/ataxia syndrome (FXTAS), beta‐propeller protein‐associated neurodegeneration (BPAN, NBIA/PARK‐WDR45), Fabry disease, Waisman syndrome, methyl CpG‐binding protein 2 (MeCP2) spectrum disorder, phosphoglycerate kinase‐1 deficiency syndrome (PGK1) and X‐linked parkinsonism and spasticity (XPDS). All clinical and radiological features reported in the literature have been reviewed. Epilepsy occasionally represents the symptom of onset, predating parkinsonism even by a few years; action tremor is another common feature along with akinetic‐rigid parkinsonism. A focus on the genetic background and its pathophysiological implications is provided. The pathogenesis of these disorders ranges from well‐defined metabolic alterations (PGK1) to non‐specific lysosomal dysfunctions (XPDS) and vesicular trafficking alterations (Waisman syndrome). However, in other cases it still remains poorly defined. Recognition of the phenotypic and genetic heterogeneity of X‐linked parkinsonism has important implications for diagnosis, management, and genetic counseling. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society
Progressive cerebellar atrophy in a patient with complex II and III deficiency and a novel deleterious variant in SDHA: A Counseling Conundrum
Authors: Sturrock Beattie R. H. , Macnamara Ellen F. , McGuire Peter , Kruk Shannon , Yang Ivan , Murphy Jennifer ,   Undiagnosed Diseases Network, Tifft Cyndi J. , Gordon‐Lipkin Eliza
Published: May 2021

Background

Complex II is an essential component of the electron transport chain, linking it with the tricarboxylic acid cycle. Its four subunits are encoded in the nuclear genome, and deleterious variants in these genes, including SDHA (OMIM 600857), are associated with a wide range of symptoms including neurological disease, cardiomyopathy, and neoplasia (paraganglioma‐pheochromocytomas (PGL/PCC), and gastrointestinal stromal tumors). Deleterious variants of SDHA are most frequently associated with Leigh and Leigh‐like syndromes.

Methods and Results

Here, we describe a case of a 9‐year‐old boy with tremor, nystagmus, hypotonia, developmental delay, significant ataxia, and progressive cerebellar atrophy. He was found to have biallelic variants in SDHA, a known pathogenic variant (c.91C>T (p.R31*)), and a variant of unknown significance (c.454G>A (p.E152K)). Deficient activity of complexes II and III was detected in fibroblasts from the patient consistent with a diagnosis of a respiratory chain disorder.

Conclusion

We, therefore, consider whether c.454G>A (p.E152K) is, indeed, a pathogenic variant, and what implications it has for family members who carry the same variant.
The causes of Fanconi anemia in South Asia and the Middle East: A case series and review of the literature
Authors: Thompson Ashley S. , Saba Nusrat , McReynolds Lisa J. , Munir Saeeda , Ahmed Parvez , Sajjad Sumaira , Jones Kristine , Yeager Meredith , Donovan Frank X. , Chandrasekharappa Settara C. , Alter Blanche P. , Savage Sharon A. , Rehman Sadia
Published: May 2021

Background

Fanconi anemia (FA) is an inherited bone marrow failure syndrome associated with characteristic dysmorphology primarily caused by biallelic pathogenic germline variants in any of 22 different DNA repair genes. There are limited data on the specific molecular causes of FA in different ethnic groups.

Methods

We performed exome sequencing and copy number variant analyses on 19 patients with FA from 17 families undergoing hematopoietic cell transplantation evaluation in Pakistan. The scientific literature was reviewed, and we curated germline variants reported in patients with FA from South Asia and the Middle East.

Results

The genetic causes of FA were identified in 14 of the 17 families: seven FANCA, two FANCC, one FANCF, two FANCG, and two FANCL. Homozygous and compound heterozygous variants were present in 12 and two families, respectively. Nine families carried variants previously reported as pathogenic, including two families with the South Asian FANCL founder variant. We also identified five novel likely deleterious variants in FANCA, FANCF, and FANCG in affected patients.

Conclusions

Our study supports the importance of determining the genomic landscape of FA in diverse populations, in order to improve understanding of FA etiology and assist in the counseling of families.
Hyperhaemolysis in a pregnant woman with a homozygous β0‐thalassemia mutation and two genetic modifiers
Authors: Jiwu Lou , Manna Sun , Lai Meixiang , Ying Zhao , Yanhui Liu
Published: May 2021

Introduction

Patients with a homozygous β0‐thalassemia mutation usually have a transfusion‐dependent β‐thalassemia major phenotype. However, some β‐thalassemia patients present with a relatively mild and even normal phenotype and always have a high level of Hb F induced by genetic modifiers.

Methods

In this study, we identified a homozygous β0‐thalassemia mutation (HBB: c.126_129delCTTT) in a 36‐year‐old pregnant woman. She had not presented any clinical symptoms of β‐thalassemia since birth. To investigate her unexpected mild phenotype, known genetic modifiers that ameliorate the severity of β‐thalassemia were analysed. Besides, we described the haematological changes during pregnancy.

Results

Two genetic modifiers (a heterozygous KLF1: c.519_525dup mutation; and two homozygous HBS1L‐MYB locus SNP variants: rs7776054 and rs9399137) were identified. However, she showed a gradually decreased level of Hb during pregnancy, and serious transfusion complication of hyperhaemolysis was induced and complicated the pregnancy.

Conclusion

This report is in accordance with previous findings that genetic modifiers can ameliorate the clinical severity of β‐thalassemia, even without obvious clinical symptoms in a prolonged steady state. However, the steady state can be disrupted during pregnancy. In addition, raising awareness of hyperhaemolysis among clinicians treating patients with thalassemia is necessary.
Prelaminated flaps in head and neck cancer reconstructive surgery: A systematic review
Authors: Fermi Matteo , Bassano Edoardo , Molinari Giulia , Alicandri‐Ciufelli Matteo , Scarpa Alfonso , Presutti Livio , De Santis Giorgio , Mattioli Francesco
Published: May 2021

Background

Prelamination is a reconstructive technique providing fasciomucosal or composite flaps with low donor‐site morbidity. We conducted a systematic review of retrospective studies to assess the application of prelaminated flaps in reconstructive surgery of head and neck cancer patients, and to evaluate the advantages and disadvantages of this technique.

Methods

This systematic review adhered to the recommendations of the PRISMA (Preferred Reporting Items of Systematic Reviews and Meta‐analysis) 2009 guidelines. A computerized MEDLINE search was performed using the PubMed service of the U.S. National Library of Medicine (www.pubmed.org) and Scopus database (www.scopus.com), running the following search string: “prelamination OR prelaminated AND flap.” Two authors screened the articles, then selected and extracted data on malignancies characteristics, reconstructive techniques, outcomes and complications.

Results

A total of 19 articles were selected and reviewed from 128 identified. Seven of 19 articles were case reports, 12 articles were case series. One‐hundred‐two patients underwent reconstructive treatment by prelamination technique using a wide variety of flaps (92 free, 10 pedicled). The sites of reconstruction were oral cavity (66 floor of the mouth, 3 retromolar trigone, 6 hard palate, 4 cheek, 4 tongue), 8 facial skin (5 of them sited on the nose), 4 oropharyngeal defects, 1 laryngotracheal region. No case of total flap loss was reported. Partial flap loss or shrinkage requiring minor surgical revisions was observed in 18 patients (17.6%). Primary closure of the donor site was achieved in 97 cases (97%) and 3 (3%) required revision surgery of the donor site.

Conclusion

Prelamination is an effective and versatile technique, with low donor‐site morbidity. Further studies would be needed to investigate the impact on the patient's oncologic outcome. More comparative studies with standard reconstructive techniques are essential to understand when it is worth performing this sophisticated procedure.
On the existence, uniqueness, and new analytic approximate solution of the modified error function in two‐phase Stefan problems
Authors: Bougoffa Lazhar , Rach Randolph C. , Mennouni Abdelaziz
Published: May 2021

This paper provides a new proof of the existence and uniqueness of the solution for a nonlinear boundary value problem

which describes the study of two‐phase Stefan problems on the semi‐infinite line [0, ∞). This result considerably extends the analysis of a recent work. A highly accurate analytic approximate solution of this problem is also provided via the Adomian decomposition method.
Fractional type multivariate neural network operators
Authors: Kadak Uğur
Published: May 2021

In this paper, we introduce a novel family of multivariate neural network operators involving Riemann‐Liouville fractional integral operator of order α. Their pointwise and uniform approximation results are presented, and new results concerning the rate of convergence in terms of the modulus of continuity are estimated. Moreover, several graphical and numerical results are presented to demonstrate the accuracy, applicability, and efficiency of the operators through special activation functions. Finally, an illustrative real‐world example on the recent trend of novel corona virus Covid‐19 has been investigated in order to demonstrate the modeling capabilities of the proposed neural network operators.
A novel lens antenna with wide beamwidth using three dimensional printing
Authors: Wang Tao , Huang Kama , Zhang Bing , Liu Wenbo , Zhang Zhuoyue , Hou Jing , Rasool Nouman
Published: May 2021

In this article, a novel lens antenna with wide beamwidth using three‐dimensional printing is proposed. The proposed antenna is comprised of a concave lens by using three‐dimensional printing, a lantern shaped patch with a parasitic ring, and eight loaded monopoles around the center. The antenna operating frequency is 5.8 GHz, and the prototype is implemented. The measured half power beamwidth are 142° and 130° at the xoz and the yoz planes, respectively. Besides, the antenna is low cost, easy to fabricated, and very low cross polarization. Measured results agree well with the simulation results.
Photonically integrated gain‐switched lasers for optical frequency comb generation
Authors: Hammad Mohab N. , Lakshmijayasimha Prajwal D. , Kaszubowska‐Anandarajah Aleksandra , Anandarajah Prince M.
Published: May 2021

The authors present the complete characterization of two cost‐efficient photonically integrated slotted lasers that offer regrowth free manufacturing. Furthermore, the application of these devices, as multi‐carrier transmitters is also investigated. In particular, the authors employ gain switching, an optical frequency comb (OFC) generation technique based on the direct modulation. The first device used is a dual section discrete mode index patterned device. It comprises two sections, implemented in a master–slave configuration to achieve optical injection locking (OIL) of the slave section. The second, a four‐section device, consists of two regrowth‐free Fabry‐Perot lasers, also forming a master–slave configuration. The authors demonstrate that the additional sections give finer control over the OIL parameters of the device. Results show that the OIL leads to the enhancement of the modulation bandwidth, which in turn can be used to generate a broader OFC. The generated OFC is characterized to demonstrate additional improvements brought by OIL such as reduced optical linewidth and relative intensity noise. The lower fabrication cost of such photonically integrated slotted lasers, accompanied by their applicability to OFC generation make them attractive potential candidates for the employment in next generation short reach optical networks.
Effects of hatching enzymes on egg envelope digestion in the male‐brooding seahorse
Authors: Zhang Huixian , Zhang Bo , Kawaguchi Mari , Lin Qiang
Published: May 2021

In the present study, we aimed to evaluate the effects of hatching enzymes on the egg envelope digestion during the hatching period in the male brooding seahorse. The complementary DNAs encoding two hatching‐enzyme genes, high choriolytic enzyme (HCE) and low choriolytic enzyme (LCE), were cloned and functionally characterized from the lined seahorse (Hippocampus erectus). The genomic‐synteny analysis confirmed that teleosts shared LCE gene synteny. In contrast, the genomic location of HCE was found to be conserved with pipefish, but not other teleosts, suggesting that translocation into a novel genomic location occurred. Whole‐mount in situ hybridization showed that HCE and LCE mRNAs were expressed in hatching gland cells. To determine the digestion mechanisms of HCE and LCE in hatching, recombinant HCE and LCE were generated and their enzyme activities were examined using fertilized egg envelopes and synthetic peptides. Seahorse HCE and LCE independently digested and softened the egg envelopes of the lined seahorse. Although the egg envelope was digested more following HCE and LCE co‐treatment, envelope solubilization was not observed. Indeed, both HCE and LCE showed similar substrate specificities toward four different synthetic peptides designed from the cleavage sites of egg envelope proteins. HCE and LCE proteins from other euteleostean fishes showed different specificities, and the egg envelope was solubilized by the cooperative action of HCE and LCE. These results suggest that the function of LCE was degenerated in the lined seahorse. Our results imply a digestion mechanism for evolutionary adaptation in ovoviviparous fish with male pregnancy.
Telemedicine visits in myasthenia gravis: Expert guidance and the Myasthenia Gravis Core Exam (MG‐CE)
Authors: Guidon Amanda C. , Muppidi Srikanth , Nowak Richard J. , Guptill Jeffrey T. , Hehir Michael K. , Ruzhansky Katherine , Burton Leeann B. , Post David , Cutter Gary , Conwit Robin , Mejia Nicte I. , Kaminski Henry J. , Howard James F.
Published: May 2021

Introduction/Aims

Telemedicine may be particularly well‐suited for myasthenia gravis (MG) due to the disorder's need for specialized care, its hallmark fluctuating muscle weakness, and the potential for increased risk of virus exposure among patients with MG during the coronavirus disease 2019 (COVID‐19) pandemic during in‐person clinical visits. A disease‐specific telemedicine physical examination to reflect myasthenic weakness does not currently exist.

Methods

This paper outlines step‐by‐step guidance on the fundamentals of a telemedicine assessment for MG. The Myasthenia Gravis Core Exam (MG‐CE) is introduced as a MG‐specific, telemedicine, physical examination, which contains eight components (ptosis, diplopia, facial strength, bulbar strength, dysarthria, single breath count, arm strength, and sit to stand) and takes approximately 10 minutes to complete.

Results

Pre‐visit preparation, remote ascertainment of patient‐reported outcome scales and visit documentation are also addressed.

Discussion

Additional knowledge gaps in telemedicine specific to MG care are identified for future investigation.
Challenges, fabrications and horizons of oxide solid electrolytes for solid‐state lithium batteries
Authors: Wei Ran , Chen Shaojie , Gao Tianyi , Liu Wei
Published: May 2021

Solid electrolyte is a key component for all‐solid‐state lithium battery that is one of the most promising technologies for next‐generation energy storages. This review describes the challenges and strategies, preparation methods and outlook of oxide solid electrolytes for solid‐state lithium batteries. The general strategies on enhancing ionic conductivity of oxide solid electrolytes and reducing impedance interface are first summarized. We then introduce the basic structures of typical oxide electrolytes. The preparation technologies are also introduced for oxide electrolytes with various dimensions including bulk, thin film and fiber. In addition, the integration of oxide electrolytes with electrode materials is highlighted. In the last part, an outlook for the future development of oxide electrolytes towards practical applications is provided.