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Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation

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Hepatobiliary & Pancreatic Diseases International 2021年第4期20卷 323-329页

作者： Adriano-Valerio Schettini Laura Llado JulieK Heimbach JoseGonzalez Costello Marie Tranäng olivier van Caenegem Richard C Daly Peter van den bergh Carlos Casasnovas Joan Fabregat John J Poterucha Maxime Foguenne Bo Göran Ericzon Jan Lerut Institute for Experimental and Clinical Research(IREC) Catholic University of Louvain(UCL)Avenue Hippocrate 55Brussels 1200Belgium Liver Transplant Unit Department of SurgeryHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Transplantation Surgery William J.von Liebig Transplant CenterMayo Clinic2001st St SWRochesterMN 55905USA Advanced Heart Failure and Transplant Unit Cardiology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Familial Amyloidotic Polyneuropathy World Transplant Registry(FAPWTR) Division of Transplantation SurgeryKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden Cardiothoracic Intensive Care Unit Department of Cardiovascular DiseasesUniversity Hospitals Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Cardiovascular Surgery Department Mayo Clinic2001st St SWRochesterMN 55905USA Neuromuscular Reference Centre University Hospital Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Neuromuscular Unit Neurology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Gastroenterology and Hepatology Mayo Clinic2001st St SWRochester MN 08907USA Division of Transplantation Surgery CLINTECKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden

Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.

关键词： Hereditary transthyretin amyloidosis Heart transplantation Liver transplantation Non-Val30Met mutation Val122del mutation Domino liver transplantation

Deciphering fluid inclusions in high-grade rocks

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Geoscience Frontiers 2014年第5期5卷 683-695页

作者： Alfons van den Kerkhof Andreas Kronz Klaus Simon Geowissenschaftliches Zentrum der Universitat Gottingen Goldschmidtstr.1-3 D 37073 GottingenGermany

The study of fluid inclusions in high-grade rocks is especially challenging as the host minerals have been normally subjected to deformation, recrystallization and fluid-rock interaction so that primary in- clusions, formed at the peak of metamorphism are rare. The larger part of the fluid inclusions found in metamorphic minerals is typically modified during uplift. These late processes may strongly disguise the characteristics of the ＂original＂ peak metamorphic fluid. A detailed microstructural analysis of the host minerals, notably quartz, is therefore indispensable for a proper interpretation of fluid inclusions. Cathodoluminescence （CL） techniques combined with trace element analysis of quartz （EPMA, LA- [CPMS） have shown to be very helpful in deciphering the rock-fluid evolution. Whereas high-grade metamorphic quartz may have relatively high contents of trace elements like Ti and A1, low- temperature re-equilibrated quartz typically shows reduced trace element concentrations. The result- ing microstructures in CL can be basically distinguished in diffusion patterns （along microfractures and grain boundaries）, and secondary quartz formed by dissolution-reprecipitation. Most of these textures are formed during retrograde fluid-controlled processes between ca. 220 and 500 ℃, i.e. the range of semi-brittle deformation （greenschist-facies） and can be correlated with the fluid inclusions. In this way modified and re-trapped fluids can be identified, even when there are no optical features observed under the microscope.

关键词： Fluid inclusionsGranuliteCharnockiteCathodoluminescenceLA-ICPMSQuartz

Quantitative resistance against Bemisia tabaci in Solanum pennellii:Genetics and metabolomics

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Journal of Integrative Plant Biology 2016年第4期58卷 397-412页

作者： Floor van den oever-van den Elsen Alejandro F.Lucatti Sjaak van Heusden Colette Broekgaarden Roland Mumm Marcel Dicke Ben Vosman Wageningen UR Plant Breeding Wageningen University and Research CentreP.O.Box 3866700AJWageningenThe Netherlands Laboratory of Entomology Wageningen University and Research CentreP.O.Box 166700AAWageningenThe Netherlands Graduate School Experimental Plant Sciences Droevendaalsesteeg 16708 PB WageningenThe Netherlands Plant Research International Business Unit BioscienceWageningen University and Research CentreP.O.Box 166700AA WageningenThe Netherlands

The whitefly Bemisia tabaci is a serious threat in tomato cultivation worldwide as all varieties grown today are highly susceptible to this devastating herbivorous *** accessions of the tomato wild relative Solanum pennellii show a high resistance towards B. tabaci. A mapping approach was used to elucidate the genetic background of whiteflyresistance related traits and associated biochemical traits in this species. Minor quantitative trait loci（QTLs） for whitefly adult survival（AS） and oviposition rate（oR） were identified and some were confirmed in an F2BC1 population, where they showed increased percentages of explained variance（more than 30%）. Bulked segregant analyses on pools of whiteflyresistant and-susceptible F2 plants enabled the identification of metabolites that correlate either with resistance or susceptibility. Genetic mapping of these metabolites showed that a large number of them co-localize with whiteflyresistance QTLs. Some of these whitefly-resistance QTLs are hotspots for metabolite QTLs. Although a large number of metabolite QTLs correlated to whitefly resistance or susceptibility, most of them are yet unknown compounds and further studies are needed to identify the metabolic pathways and genes involved. The results indicate a direct genetic correlation between biochemical-based resistance characteristics and reduced whitefly incidence in S. pennellii.

关键词： Genetic linkage map life-history metabolic fingerprinting parameters tomato whitefly

The rediscovery of Strix butleri(Hume,1878)in oman and Iran,with molecular resolution of the identity of Strix omanensis Robb,van den Berg and Constantine,2013

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Chinese Birds 2016年第2期7卷 85-94页

作者： Magnus S.Robb George Sangster Mansour Aliabadian Arnoud B.van den Berg Mark Constantine Martin Irestedt Ali Khani Seyed Babak Musavi Joao M.G.Nunes Maia Sarrouf Willson Alyn J.Walsh The Sound Approach Department of Bioinformatics and Genetics Swedish Museum of Natural History Department of Zoology Stockholm University Department of Biology Faculty of ScienceFerdowsi University of Mashhad Research Department of Zoological Innovations Institute of Applied ZoologyFaculty of ScienceFerdowsi University of Mashhad Khorasan-e Razavi Provincial office of the Department of the Environment Khucheh Gochin Parque Ecológico do Funchal Environment Society of oman Wildfowl Reserve

Background:Many species of owls(Strigidae) represent cryptic species and their taxonomic study is in *** recent years,two new species of owls of the genus Strix have been described from the Middle East by different research *** has been suggested that one of these species,***,is not a valid species but taxonomic compari?sons have been hampered by the lack of voucher specimens of ***,and the poor state of the holotype of ***:Here we use new DNA sequence data to clarify the taxonomy and nomenclature of the *** *** also report the capture of a single *** sensu stricto in Mashhad,***:A cytochrome b sequence of *** was found to be identical to that of the holotype of ***,indicating that the name *** is best regarded as a junior synonym of *** identity of the *** captured in Mashhad,Iran,was confirmed using DNA sequence *** represents a major(1300 km) range exten?sion of this ***:The population discovered in oman in 2013 and originally named ‘***' actually represents the rediscovery of ***,which was known from a single specimen and had not been recorded since *** range of *** extends into northeast *** study augments the body of evidence for the recognition of *** and *** as separate species and highlights the importance of using multiple evidence to study cryptic owl species.

关键词： Molecular identification Nomenclature Phylogenetics Strigidae Strix Taxonomy

Pharmacologic RNA splicing modulation: a novel mechanism to enhance neoantigen-directed anti-tumor immunity and immunotherapy response

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Signal Transduction and Targeted Therapy 2021年第11期6卷 3167-3168页

作者： Kerryn Elliott Jonas Nilsson Jimmy van den Eynden Department of Medical Biochemistry and Cell Biology Institute of BiomedicineSahlgrenska Academy at University of GothenburgGothenburgSweden Sahlgrenska Cancer Center Department of SurgeryInstitute of Clinical SciencesUniversity of Gothenburg and Sahlgrenska University HospitalGothenburgSweden Harry Perkins Institute of Medical Research University of Western AustraliaPerthWAAustralia Department of Human Structure and Repair Anatomy and Embryology UnitGhent UniversityGhentBelgium

A recent study by Lu et al.1 suggests a novel approach to increasing responsiveness to immune checkpoint blockade (ICB) therapy. ICB is a promising form of cancer immunotherapy that aims to boost the anti-tumoral immune response. This response is primarily driven by the presentation of different types of antigens at the cancer cell membrane via the type I major histocompatibility complex (MHC I). Neoantigens are tumor-specific antigens that are small (mostly 9-mers) mutated peptides that generally result from somatic mutations. High tumor mutational burden, which results in the formation of many neoantigens, is currently one of the main biomarkers for ICB response prediction. Because ICB is only effective in a minority of patients, new therapeutic strategies are required to augment this response.

关键词： immunity immunotherapy mostly

Decision support model for effects estimation and proportionality assessment for targeting in cyber operations

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Defence Technology（防务技术） 2021年第2期17卷 352-374页

作者： C.Maathuis W.Pieters J.van den Berg Delft University of Technology Jaffalaan 52628 BXDelftNetherlands TNo Military operations Oude Waaldorperweg 632597 AKDen HaagNetherlands Netherlands Defense Academy De la Reyweg 1204818 BBBredaNetherlands open University of the Netherlands Valkenburgerweg 1776419 ATHeerlenNetherlands

Cyber operations are relatively a new phenomenon of the last two *** that period,they have increased in number,complexity,and agility,while their design and development have been processes well kept under *** a consequence,limited data(sets)regarding these incidents are *** various academic and practitioner public communities addressed some of the key points and dilemmas that surround cyber operations(such as attack,target identification and selection,and collateral damage),still methodologies and models are needed in order to plan,execute,and assess them in a responsibly and legally compliant *** on these facts,it is the aim of this article to propose a model that i))estimates and classifies the effects of cyber operations,and ii)assesses proportionality in order to support targeting decisions in cyber *** order to do that,a multi-layered fuzzy model was designed and implemented by analysing real and virtual realistic cyber operations combined with interviews and focus groups with technical e military *** proposed model was evaluated on two cyber operations use cases in a focus group with four technical e military *** the design and the results of the evaluation are revealed in this article.

关键词： Cyber operations Cyber warfare Cyber weapons Artificial intelligence Fuzzy logic Targeting

Combining two-photon lithography with laser ablation of sacrificial layers:A route to isolated 3D magnetic nanostructures

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Nano Research 2023年第1期16卷 1441-1447页

作者： Arjen van den Berg Mylène Caruel Matthew Hunt Sam Ladak School of Physics and Astronomy Cardiff UniversityCardiffCF243AAUK Institut National des Sciences Appliquées(INSA)of Toulouse 35 Avenue de Rangueil31400 ToulouseFrance

Three-dimensional(3D)nanostructured functional materials are important systems allowing new means for intricate control of electromagnetic properties.A key problem is realising a 3D printing methodology on the nanoscale that can yield a range of functional *** this article,it is shown that two-photon lithography,when combined with laser ablation of sacrificial layers,can be used to realise such a vision and produce 3D functional nanomaterials of complex ***-of-principle is first shown by fabricating planar magnetic nanowires raised above the substrate that exhibit controlled domain wall injection and ***,3D artificial spin-ice(3DASI)structures are fabricated,whose complex switching can be probed using optical *** show that by careful analysis of the magneto-optical Kerr effect signal and by comparison with micromagnetic simulations,depth dependent switching information can be obtained from the 3DASI *** work paves the way for new materials,which exploit additional physics provided by non-trivial 3D geometries.

关键词： three-dimensional(3D)nanomagnetism 3D lithography 3D artificial spin ice sacrificial layers magneto-optical Kerr effect

Review on modelling of corrosion under droplet electrolyte for predicting atmospheric corrosion rate

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Journal of Materials Science & Technology 2021年第3期62卷 254-267页

作者： Bangalore Gangadharacharya Koushik Nils van den Steen Mesﬁn Haile Mamme Yves van Ingelgem Herman Terryn Vrije Universiteit Brussel Research Group Electrochemical and Surface Engineering(SURF)Pleinlaan 21050 BrusselsBelgium Vrije Universiteit Brussel Eenheid Algemene Chemie(ALGC)Pleinlaan 21050 BrusselsBelgium

Atmospheric corrosion of metals is the most common type of corrosion which has a significant impact on the environment and operational safety in various situations of everyday *** of the common examples can be observed in land,water and air transportation systems,electronic circuit boards,urban and offshore *** dew drops formed on metal surface due to condensation of atmospheric moisture facilitates corrosion as an *** corrosion mechanisms under these droplets are different from classically known bulk electrolyte *** to thin and non-uniform geometric thickness of the droplet electrolyte,the atmospheric oxygen requires a shorter diffusion path to reach the metal *** corrosion under a droplet is driven by the depletion of oxygen in the center of the droplet compared to the edge,known as differential *** case of a larger droplet,differential aeration leads to preferential cathodic activity at the edge and is controlled by the droplet ***,for a smaller droplet,the oxygen concentration remains uniform and hence cathodic activity is not controlled by droplet *** geometry of condensed droplets varies dynamically with changing environmental parameters,influencing corrosion mechanisms as the droplets evolve in *** this review,various modelling approaches used to simulate the corrosion under droplet electrolytes are *** the efforts of developing a comprehensive model to estimate corrosion rates,it has been noted from this review that the influence of geometric evolution of the droplet due to condensation/evaporation processes on corrosion mechanisms are yet to be *** varying external factors like environmental temperature,relative humidity,presence of hygroscopic salts and pollutants influence the evolution of droplet electrolyte,making it a complex phenomenon to ***,an overview of available dropwise condensation and evaporation models which descri

关键词： Atmospheric corrosion Water droplets Mathematical modelling Condensation Evaporation

Atlas of oocytes, Zygotes and Embryos in Reproductive Medicine 1

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