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Andreas Brodehl Alexey Meshkov Roman Myasnikov Anna Kiseleva Olga Kulikova Brbel Klauke Evgeniia Sotnikova Caroline Stanasiuk Mikhail Divashuk Greta Marie Pohl Maria Kudryavtseva Karin Klingel Brenda Gerull Anastasia Zharikova Jan Gummert Sergey Koretskiy Stephan Schubert Elena Mershina Anna Grtner Polina Pilus Kai Thorsten Laser Valentin Sinitsyn Sergey Boytsov Oxana Drapkina Hendrik Milting 《International journal of molecular sciences》2021,22(7)
About 50% of patients with arrhythmogenic cardiomyopathy (ACM) carry a pathogenic or likely pathogenic mutation in the desmosomal genes. However, there is a significant number of patients without positive familial anamnesis. Therefore, the molecular reasons for ACM in these patients are frequently unknown and a genetic contribution might be underestimated. Here, we used a next-generation sequencing (NGS) approach and in addition single nucleotide polymor-phism (SNP) arrays for the genetic analysis of two independent index patients without familial medical history. Of note, this genetic strategy revealed a homozygous splice site mutation (DSG2–c.378+1G>T) in the first patient and a nonsense mutation (DSG2–p.L772X) in combination with a large deletion in DSG2 in the second one. In conclusion, a recessive inheritance pattern is likely for both cases, which might contribute to the hidden medical history in both families. This is the first report about these novel loss-of-function mutations in DSG2 that have not been previously identi-fied. Therefore, we suggest performing deep genetic analyses using NGS in combination with SNP arrays also for ACM index patients without obvious familial medical history. In the future, this finding might has relevance for the genetic counseling of similar cases. 相似文献
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This study was devoted to the prediction of polymer material aging. The prediction of the shelf time of tire rubber is used as an example in this article. The main steps of the whole procedure are described. They are the design of the experiment, accelerated aging testing, the construction of a multiresponse mathematical model and parameter estimation, and the extrapolation of the model in real‐life settings. The main pitfalls were deduced, and techniques to overcome these pitfalls are described. Novel methods of data modeling, such as evolutionary design of experiment and successive Bayesian estimation, were used. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 95: 1275–1284, 2005 相似文献
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The temperature and electric- and magnetic-field dependences of the resistivity of the R0.1Bi1.9Te3 compound are investigated. It is shown that, in the low-temperature region, variable-range hopping conductivity is realized in this compound. In the temperature range of hopping conductivity, the electrical resistivity decreases with increasing electric-field strength in the sample, which is typical of charge-carrier tunneling from one localized state in the impurity band to another. Investigation of the transverse magnetoresistance revealed the crossover from the parabolic dependence of the magnetoresistance in low fields to the linear dependence in high fields. The established features of the transport properties of the R0.1Bi1.9Te3 compound are characteristic of inhomogeneous and disordered semiconductors. 相似文献
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Nanopowders of Bi2Te3 and R0.1Bi1.9Te3 (where R = Er, Tm, Yb, Lu) are obtained by microwave solvothermal synthesis. The powder-like materials are compacted by cold isostatic compression followed by annealing in argon. The influence of the doping agent on the structure and characteristics of the derived materials are investigated. It is demonstrated that the introduction of rare-earth elements (2 at %) into the bismuth-telluride lattice leads to a decrease in the electrical resistivity and to an increase in the Seebeck coefficient. The best thermoelectric properties are obtained for the sample of bismuth telluride doped with thulium. 相似文献
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Taran Oxana P. Yashnik Svetlana A. Boltenkov Vadim V. Parkhomchuk Ekaterina V. Sashkina Kseniya A. Ayusheev Artemiy B. Babushkin Dmitrii E. Parmon Valentin N. 《Topics in Catalysis》2019,62(5-6):491-507
Topics in Catalysis - The process of synthesis of formic acid via partial peroxide oxidation of methane over Fe-MFI zeolites, as well as the influence of the catalyst activation by oxalic acid on... 相似文献
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Kholdeeva Oxana A. Ivanchikova Irina D. Maksimchuk Nataliya V. Melgunov Maxim S. Chang Jong-San Guidotti Matteo Shutilov Alexei A. Zaikovskii Vladimir I. 《Topics in Catalysis》2014,57(17):1377-1384
Catalytic properties of Ti-containing porous solids were compared in the oxidation of 2,3,6-trimethylphenol (TMP) with H2O2 to produce 2,3,5-trimethyl-1,4-benzoquinone (TMBQ, vitamin E key intermediate). Mesoporous titanium–silicates with di(oligo)nuclear Ti centers, metal–organic framework MIL-125 and amorphous TiO2 demonstrated 100 % selectivity toward TMBQ. Titanium–silicates prepared by evaporation-induced self-assembly revealed superior performance in terms of product yield and catalyst reusability.
相似文献8.
The effect of mechanical recycling on the microstructure and properties of PA66 composites reinforced with carbon fibers 下载免费PDF全文
Giovanna Colucci Oxana Ostrovskaya Alberto Frache Brunetto Martorana Claudio Badini 《应用聚合物科学杂志》2015,132(29)
This article aims to prepare by injection molding recycled polymeric composites based on PA66 reinforced with short carbon fibers after artificial aging for applications in the automotive field. The aging cycles involves the combined action of UV radiation, moisture, and temperature in order to simulate the common outdoor conditions. The 100% recycled composites are obtained by the regranulation of the aged specimens followed by the remelting and re‐injection molding. The study is focused on the comparison between the mechanical behavior and the microstructure of the composites before and after mechanical recycling. The results of mechanical, thermal, and morphological investigations reveal that the recycling process had no significant effect on the final properties and microstructure of the recycled composites. Therefore the recycled PA66CF30 composites could be successfully used for structural or semi‐structural automotive applications guaranteeing good final performances and advantages from the environmental point of view. © 2015 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2015 , 132, 42275. 相似文献
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Mauricio Garza Castañón S. Velumani Oxana Vasilievna Kharissova Marco A. Jiménez Arunachala M. Kannan 《国际能源研究杂志》2011,35(7):594-600
Application of tertiary alloy nanoparticles is becoming more important, however, the local structure of such alloyed particles, which is critical for tailoring their properties, is not yet very clearly understood. In this study, we present detailed theoretical analysis on the atomistic structure and CO adsorption in Pd70Co20X10 (X=Au, Mo, Ni) tertiary composite alloys for their application in fuel cells toward oxygen reduction reaction (ORR). Basic structure and the most stable configurations for all the three composites are determined. Quantum mechanical approaches and classic molecular dynamics methods are applied to model the structure and to determine the lowest energy configurations. Our theoretical results agree well with the experimental results of XRD patterns. Considering those structures as the base, simulations were performed to determine the magnitude of CO poisoning. The results obtained by ab‐initio calculations allow us to estimate the CO‐tolerance that these catalysts might have, along with those obtained for Pd‐Co‐Ni (70:20:10 atom %) tertiary alloy, and compared with commercial Pt (1 1 0) catalyst. From these results, a comparison has been made to show different CO adsorption strengths. This is the first step to fabricate an efficient engineering design that allows us to obtain high‐performance, low‐cost nanostructured catalysts. Copyright © 2010 John Wiley & Sons, Ltd. 相似文献
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Identification and chemical characterization of specific organic indicators in the effluents from chemical production sites 总被引:1,自引:0,他引:1
The structural diversity of the wastewater composition was described by the use of detailed non-target screening analyses of industrial effluents from chemical production sites. Determination of the indicative organic compounds acting as potential molecular indicators for industrial emissions from chemical production industries has been possible due to (i) detailed characterisation of industrial contaminants and identification of compounds with high source specificity, (ii) quantitative determination of the organic constituents in the industrial effluents and (iii) the review of their industrial applications. The determination of potential site-specific markers and industrial molecular indicators corresponding to certain production processes (production of starting materials for manufacturing paper and printing inks, powder coatings as well as epichlorohydrin production) was performed in this work.The results of this study allowed significant contributions to the chemical characterisation of industrial contaminants and isolation of indicators that can act as representatives of industrial effluents in the aquatic environment. 相似文献