bluuebunny/crossref_metadata_2025 huggingface dataset:
Francisco Toledo, seus inícios
Authors: Moraes Vanessa
Published: September 2020

Neste artigo, observa-se o desenvolvimento da obra de Francisco Toledo entre 1953 e 1963, rastreando aprendizagens e influências entre Oaxaca e Paris. Propõe-se, desse modo, doze apontamentos temáticos para abrir um diálogo sobre o início de sua obra gráfica.
Nelson Rodrigues e o melodrama no teatro contemporâneo: a transposição para o palco do folhetim Escravas do amor, da companhia teatral carioca Fodidos Privilegiados
Authors: Montebelo Barcelos Carolina
Published: September 2020

Esse estudo tem por objetivo examinar o modo pelo qual a companhia teatral carioca Fodidos Privilegiados, inserida no contexto do teatro contemporâneo do trabalho colaborativo, transpôs o folhetim Escravas do amor para o palco, intensificando cenicamente os elementos melodramáticos presentes no texto original. Através de recursos teatrais próprios da estética da companhia – uso de números coreografados, quadro vivo, narração, economia de cenário e objetos cênicos-, que são utilizados em quase todas as suas peças, a montagem de Escravas do amor é uma adaptação do folhetim de 530 páginas escrito por Nelson Rodrigues sob o pseudônimo de Suzana Flag. Desse modo, procura-se analisar como os Fodidos Privilegiados encenaram características do folhetim, quais sejam, construção em abismo, embuste e ardil, locais relacionados à intriga (choupana, floresta, albergue e castelo) e personagens como o traidor, o justiceiro, a vítima e o bobo, através de elementos melodramáticos como uso da música, mímica facial e corporal, gestos grandes e uso exacerbado da voz. Como aporte teórico no que diz respeito ao melodrama e folhetim, são utilizados estudos de Silvia Oroz, Marlyse Meyer e Jesús Martín-Barbero.
VP 18 Stef Scagliola note 18 Galuh Wandita 2012
Authors: scagliola stef
Published: September 2020

Ingesproken herinnering aan een ontmoeting en gesprek op een congres in Australie in februari 2012 over Historical Injustices
VP 20 Ibu Wanti.Sedari
Authors: Immler Nicole L. , Wanti Ibu
Published: September 2020

Fragment uit interview met Ibu Wanti, zie: https://easy.dans.knaw.nl/ui/datasets/id/easy-dataset:99985
VP 30 Groepsinterview Tati Krysnawati
Authors: Immler Nicole L. , Krisnawati Tati , Liem Suzanne , Yahuti Ramyakim Aji , ter Weel Paul
Published: September 2020

Notes and light edited transcript of a conversation that Nicole Immler had with the owners of a biological rice farm in the village of Pasiwari, very close to Rawagede. Tati Krisnawati (Indonesian social worker, works for a NGO) en Paul ter Weel (Dutch agronomist who runs the

biological rice farm).Tati’s family comes from the village they live in, neighbouring Rawagede. Also present were photographer Suzanne Liem and Interpreter Aji Yahuti Ramyakim.
VP 42 Interview Jeffry Pondaag
Authors: Immler Nicole L.
Published: September 2020

Nicole Immler - fragment uit interview zie https://easy.dans.knaw.nl/ui/datasets/id/easy-dataset:100751
VP 44 Sukarman Interview
Authors: Immler Nicole L.
Published: September 2020

Nicole Immler - fragment uit gedeponeerd interview zie DANS

https://easy.dans.knaw.nl/ui/datasets/id/easy-dataset:99841
Mid-level clouds are frequent above the southeast Atlantic stratocumulus clouds
Authors: Adebiyi Adeyemi A. , Zuidema Paquita , Chang Ian , Burton Sharon P. , Cairns Brian
Published: September 2020

Abstract. Shortwave-absorbing aerosols seasonally overlay extensive low-level
stratocumulus clouds over the southeast Atlantic. While much attention has
focused on the interactions between the low-level clouds and the overlying
aerosols, few studies have focused on the mid-level clouds that also occur
over the region. The presence of mid-level clouds over the region
complicates the space-based remote-sensing retrievals of cloud properties
and the evaluation of cloud radiation budgets. Here we characterize the
mid-level clouds over the southeast Atlantic using lidar- and radar-based
satellite cloud retrievals and observations collected in September 2016
during the ORACLES (ObseRvations of Aerosols above CLouds and their
intEractionS) field campaign. We find that mid-level clouds over the
southeast Atlantic are relatively common, with the majority of the clouds
occurring between altitudes of 5 and 7 km and at temperatures between 0 and −20 ∘C. The mid-level clouds occur at the top of a moist mid-tropospheric
smoke-aerosol layer, most frequently between August and October, and closer
to the southern African coast than farther offshore. They occur more
frequently during the night than during the day. Between July and October,
approximately 64 % of the mid-level clouds had a geometric cloud
thickness less than 1 km, corresponding to a cloud optical depth of less
than 4. A lidar-based depolarization–backscatter relationship for September
2016 indicates that the mid-level clouds are liquid-only clouds with no
evidence of the existence of ice. In addition, a polarimeter-derived cloud
droplet size distribution indicates that approximately 85 % of the
September 2016 mid-level clouds had an effective radius less than 7 µm, which could further discourage the ability of the clouds to glaciate.
These clouds are mostly associated with synoptically modulated
mid-tropospheric moisture outflow that can be linked to the detrainment from
the continental-based clouds. Overall, the supercooled mid-level clouds
reduce the radiative cooling rates of the underlying low-altitude cloud tops
by approximately 10 K d−1, thus influencing the regional cloud radiative
budget.
Attribution of the accelerating increase in atmospheric methane during 2010–2018 by inverse analysis of GOSAT observations
Authors: Zhang Yuzhong , Jacob Daniel J. , Lu Xiao , Maasakkers Joannes D. , Scarpelli Tia R. , Sheng Jian-Xiong , Shen Lu , Qu Zhen , Sulprizio Melissa P. , Chang Jinfeng , Bloom Anthony A. , Ma Shuang , Worden John , Parker Robert J. , Boesch Hartmut
Published: September 2020

Abstract. We conduct a global inverse analysis of 2010–2018 GOSAT satellite observations to better understand the factors controlling atmospheric methane and its accelerating increase over the 2010–2018 period. The inversion optimizes 2010–2018 anthropogenic methane emissions and their trends on a 4º × 5º grid, monthly regional wetland emissions, and annual hemispheric concentrations of tropospheric OH (the main sink of methane) also for individual years. We use an analytical solution to the Bayesian optimization problem that provides closed-form estimates of error covariances and information content for the solution. Our inversion successfully reduces the errors against the independent methane observations from the TCCON network and reproduces the interannual variability of the methane growth rate inferred from NOAA background sites. We find that prior estimates of fuel-related emissions reported by individual countries to the United Nations are too high for China (coal) and Russia (oil/gas), and too low for Venezuela (oil/gas) and the U.S. (oil/gas). We show that the 2010–2018 increase in global methane emissions is mainly driven by tropical wetlands (Amazon and tropical Africa), boreal wetlands (Eurasia), and tropical livestock (South Asia, Africa, Brazil), with no significant trend in oil/gas emissions. While the rise in tropical livestock emissions is consistent with bottom-up estimates of rapidly growing cattle populations, the rise in wetland emissions needs to be better understood. The sustained acceleration of growth rates in 2016–2018 relative to 2010–2013 is mostly from wetlands, while the peak methane growth rates in 2014–2015 are also contributed by low OH concentrations (2014) and high fire emissions (2015). Our best estimate is that OH did not contribute significantly to the 2010–2018 methane trend other than the 2014 spike, though error correlation with global anthropogenic emissions limits confidence in this result.
Calibration of global MODIS cloud amount using CALIOP cloud profiles
Authors: Kotarba Andrzej Z.
Published: September 2020

Abstract. The Moderate Resolution Imaging Spectroradiometer (MODIS) cloud detection procedure classifies instantaneous fields of view (IFOVs) as
either “confident clear”, “probably clear”, “probably cloudy”, or “confident cloudy”. The cloud amount calculation requires quantitative cloud fractions to be assigned to these classes. The operational procedure used by the MODIS Science Team assumes that confident clear and probably clear IFOVs are cloud-free (cloud fraction 0 %), while the remaining categories are completely filled with clouds (cloud fraction 100 %). This study demonstrates that this “best-guess” approach is unreliable, especially on a regional/local scale. We use data from the Cloud-Aerosol Lidar with Orthogonal Polarization (CALIOP) instrument flown on the Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observation (CALIPSO) mission, collocated with Aqua MODIS IFOV. Based on 33 793 648 paired observations acquired in January and July 2015, we conclude that actual cloud fractions to be associated with MODIS cloud mask categories are 21.5 %, 27.7 %, 66.6 %, and 94.7 %. Spatial variability is significant, even within a single MODIS algorithm path, and the operational approach introduces uncertainties of up to 30 % of cloud amount, notably in polar regions at night, and in selected locations over the Northern Hemisphere (e.g. China, the north-west coast of Africa, and eastern parts of the United States). Consequently, applications of MODIS data on a regional/local scale should first assess the extent of the uncertainty. We suggest using CALIPSO-based cloud fractions to improve MODIS cloud amount estimates. This approach can also be used for Terra MODIS data, and other passive cloud imagers, where the footprint is collocated with CALIPSO.
Increase in ocean acidity variability and extremes under increasing atmospheric CO2
Authors: Burger Friedrich A. , John Jasmin G. , Frölicher Thomas L.
Published: September 2020

Abstract. Ocean acidity extreme events are short-term periods of relatively high [H+] concentrations. The uptake of anthropogenic CO2 emissions by the ocean is expected to lead to more frequent and intense ocean acidity extreme events, not only due to changes in the long-term mean but also due to changes in short-term variability. Here, we use daily mean output from a five-member ensemble simulation of a comprehensive Earth system model under low- and high-CO2-emission scenarios to quantify historical and future changes in ocean acidity extreme events. When defining extremes relative to a fixed preindustrial baseline, the projected increase in mean [H+] causes the entire surface ocean to reach a near-permanent acidity extreme state by 2030 under both the low- and high-CO2-emission scenarios. When defining extremes relative to a shifting baseline (i.e., neglecting the changes in mean [H+]), ocean acidity extremes are also projected to increase because of the simulated increase in [H+] variability; e.g., the number of days with extremely high surface [H+] conditions is projected to increase by a factor of 14 by the end of the 21st century under the high-CO2-emission scenario relative to preindustrial levels. Furthermore, the duration of individual extreme events is projected to triple, and the maximal intensity and the volume extent in the upper 200 m are projected to quintuple. Similar changes are projected in the thermocline. Under the low-emission scenario, the increases in ocean acidity extreme-event characteristics are substantially reduced. At the surface, the increases in [H+] variability are mainly driven by increases in [H+] seasonality, whereas changes in thermocline [H+] variability are more influenced by interannual variability. Increases in [H+] variability arise predominantly from increases in the sensitivity of [H+] to variations in its drivers (i.e., carbon, alkalinity, and temperature) due to the increase in oceanic anthropogenic carbon. The projected increase in [H+] variability and extremes may enhance the risk of detrimental impacts on marine organisms, especially for those that are adapted to a more stable environment.
Consumption of safe drinking water in Pakistan: its dimensions and determinants
Authors: Akram Naeem
Published: September 2020

Abstract. Safe drinking water is one of the basic human needs. Poor quality of
drinking water is directly associated with various waterborne diseases. The
present study has attempted to analyze the household preferences for
drinking water sources and the adoption of household water treatment (HWT)
in Pakistan by using the household data of Pakistan Demographic and Health
Survey 2017–2018 (PDHS, 2018). This study found that people living in rural areas, those with older heads of household and those with large family sizes are significantly less likely
to use water from bottled or filtered water. Households with media
exposure, education, women's empowerment in household purchases and high incomes are more likely to use bottled or filtered
water. Similarly, households are more likely to adopt HWT in urban areas, when there is a higher level of awareness (through education and media), higher incomes, women enjoy a higher level of empowerment, and piped water is already used. However, households that use water from wells and have higher family sizes are less likely to adopt water purifying methods at home.
Simulating the Early Holocene demise of the Laurentide Ice Sheet with BISICLES (public trunk revision 3298)
Authors: Matero Ilkka S. O. , Gregoire Lauren J. , Ivanovic Ruza F.
Published: September 2020

Abstract. Simulating the demise of the Laurentide Ice Sheet covering Hudson Bay in the Early Holocene (10–7 ka) is important for understanding the role of accelerated changes in ice sheet topography and melt in the 8.2 ka event, a century long cooling of the Northern Hemisphere by several degrees.
Freshwater released from the ice sheet through a surface mass balance instability (known as the saddle collapse) has been suggested as a major forcing for the 8.2 ka event, but the temporal evolution of this pulse has not been constrained.
Dynamical ice loss and marine interactions could have significantly accelerated the ice sheet demise, but simulating such processes requires computationally expensive models that are difficult to configure and are often impractical for simulating past ice sheets.
Here, we developed an ice sheet model setup for studying the Laurentide Ice Sheet's Hudson Bay saddle collapse and the associated meltwater pulse in unprecedented detail using the BISICLES ice sheet model, an efficient marine ice sheet model of the latest generation which is capable of refinement to kilometre-scale resolutions and higher-order ice flow physics.
The setup draws on previous efforts to model the deglaciation of the North American Ice Sheet for initialising the ice sheet temperature, recent ice sheet reconstructions for developing the topography of the region and ice sheet, and output from a general circulation model for a representation of the climatic forcing.
The modelled deglaciation is in agreement with the reconstructed extent of the ice sheet, and the associated meltwater pulse has realistic timing. Furthermore, the peak magnitude of the modelled meltwater equivalent (0.07–0.13 Sv) is compatible with geological estimates of freshwater discharge through the Hudson Strait. The results demonstrate that while improved representations of the glacial dynamics and marine interactions are key for correctly simulating the pattern of Early Holocene ice sheet retreat, surface mass balance introduces by far the most uncertainty. The new model configuration presented here provides future opportunities to quantify the range of plausible amplitudes and durations of a Hudson Bay ice saddle collapse meltwater pulse and its role in forcing the 8.2 ka event.
Development of an atmospheric chemistry model coupled to the PALM model system 6.0: Implementation and first applications
Authors: Khan Basit , Banzhaf Sabine , Chan Edward C. , Forkel Renate , Kanani-Sühring Farah , Ketelsen Klaus , Kurppa Mona , Maronga Björn , Mauder Matthias , Raasch Siegfried , Russo Emmanuele , Schaap Martijn , Sühring Matthias
Published: September 2020

Abstract. In this article we describe the implementation of an online-coupled gas-phase chemistry model in the turbulence resolving PALM model system 6.0. The new chemistry model is part of the PALM-4U components (read: PALM for you; PALM for urban applications) which are designed for application of PALM model in the urban environment (Maronga et al., 2020). The latest version of the Kinetic PreProcessor (KPP, 2.2.3), has been utilised for the numerical integration of gas-phase chemical reactions. A number of tropospheric gas-phase chemistry mechanisms of different complexity have been implemented ranging from the photostationary state to more complex mechanisms such as CBM4, which includes major pollutants namely O3, NO, NO2, CO, a simplified VOC chemistry and a small number of products. Further mechanisms can also be easily added by the user. In this work, we provide a detailed description of the chemistry model, its structure along with its various features, input requirements, its application and limitations. A case study is presented to demonstrate the application of the new chemistry model in the urban environment. The computation domain of the case study is comprised of part of Berlin, Germany, covering an area of 6.71 × 6.71 km with a horizontal resolution of 10 m. We used "PARAMETERIZED" emission mode of the chemistry model that only considers emissions from traffic sources. Three chemical mechanisms of varying complexity and one no-reaction (passive) case have been applied and results are compared with observations from two permanent air quality stations in Berlin that fall within the computation domain. The results show importance of online photochemistry and dispersion of air pollutants in the urban boundary layer. The simulated NOx and O3 species show reasonable agreement with observations. The agreement is better during midday and poorest during the evening transition hours and at night. CBM4 and SMOG mechanisms show better agreement with observations than the steady state PHSTAT mechanism.
Spatial and temporal variations in snow chemistry along a traverse from coastal East Antarctica to the ice sheet summit (Dome A)
Authors: Shi Guitao , Ma Hongmei , Hu Zhengyi , Chen Zhenlou , An Chunlei , Jiang Su , Li Yuansheng , Ma Tianming , Yu Jinhai , Wang Danhe , Lu Siyu , Sun Bo , Hastings Meredith G.
Published: September 2020

Abstract. There is a large variability in environmental conditions across the Antarctic ice sheet, and it is of significance to investigate the snow chemistry at as many locations as possible and over time, given that the ice sheet itself, and precipitation and deposition patterns and trends are changing. The China inland Antarctic traverse from coastal Zhongshan Station to the ice sheet summit (Dome A) covers a variety of environments, allowing for a vast collection of snow chemistry conditions across East Antarctica. Surface snow and snow pit samples were collected on this traverse during five campaigns, to comprehensively investigate the spatial and temporal variations in chemical ions (Cl−, NO3−, SO42−, Na+, NH4+, K+, Mg2+, and Ca2+) and the related controlling factors. Results show that spatial patterns of ions in surface snow are consistent among the five campaigns, with Cl−, Na+, K+, and Mg2+ decreasing rapidly with distance from the coast and NO3− showing an opposite pattern. No clear spatial trends in SO42−, NH4+ and Ca2+ were found. In the interior areas, an enrichment of Cl− versus Na+ with respect to seawater composition is ubiquitous as a result of the deposition of HCl, which can account for up to ~40 % of the total Cl− budget, while enriched K+ and Mg2+ are associated with terrestrial particle mass. Ca2+ and SO42− in surface snow are significantly enriched relative to Na+, related to terrestrial dust inputs and marine biogenic emissions, respectively. Snow NH4+ is mainly associated with marine biological activities, with higher concentrations in summer than in winter. On the coast, parts of the winter snow are characterized with a depletion of SO42− versus Na+, and a significant negative correlation between nssSO42− and Na+ was found, suggesting that sea salts originated from the sea ice. In the interior areas, the negative nssSO42− signal in winter snow resulted from inputs of sea salts being completely swamped by the contribution of marine biogenic emissions. Ternary plots of Cl−, Na+, and SO42− suggest that sea salt modification is generally negligible on the coast, while the degree of modification processes to sea salts is high in the interior areas, especially during the summertime. Ion flux assessment suggests an efficient transport of nssSO42− to at least as far inland as the ~2800 m contour line. The interannual variations in ion concentrations in surface snow on the traverse are likely linked to the changes in the Southern Indian Ocean low (SIOL) from year to year, and the deepening of the SIOL in summer tends to promote the transport of marine aerosols to Princess Elizabeth Land.
Diversity, Inclusion, and Professionalism in Japanese Language Education: Introduction to the Special Section
Authors: Mori Junko , Hasegawa Atsushi
Published: September 2020

Diversity and inclusion have become a major concern in academic and professional institutions in recent years. As educators, we are responsible for creating environments where a diverse population of students can communicate beyond differences and learn from each other. While this educational mission is widely recognized, we have not sufficiently examined the extent to which a culture of diversity and inclusion has been fostered and actually practiced within our professional community. The current special section aims to facilitate dialogs on this topic among Japanese-language educators by sharing the results of an online survey conducted in 2018 and featuring commentaries prepared by twelve individuals who have contributed to Japanese language education in North America in different capacities. This introductory articleprovides a brief overview of the backgrounds and motivations for this special section and outlines its organization.
Language Ideology and Its Manifestations: Exploring Implications for Japanese Language Teaching
Authors: Bhattacharya Mahua
Published: September 2020

Language teaching is often seen as an ideologically neutral activity. Linguists have traditionally believed that what people say about language use or structure does not represent ‘real’ linguistic data (Schieffelin, et al, 1998:11).  However, it is precisely this dismissal that modern linguistic anthropologists hope to dispel. This paper attempts to lay bare the workings of language ideology and how it impacts language teaching in general and Japanese language pedagogy in particular.The ideological orientation of what constitutes ‘standard’ Japanese language involves inclusion of certain components that are motivated by Nihonjinron discourses of ‘identity, aesthetics, morality and epistemology’ and processes of exclusion that ‘erase’ deviations from the ‘norm’ (Schieffelin, et al, 1998:3). Ideas about ‘native speaker’ understanding, selection of language materials, inclusion and exclusion of syntactical, lexical, and pragmatic forms in teaching manuals, etc., are all affected by these perspectives, some of which this paper will hope to enumerate. With concrete examples it will be demonstrated how flawed these processes are and how a critical pedagogical approach may help solve these issues.
Priority Areas for Water Resources Conservation: Study Case Canal Guandu Watershed
Authors: Vieira Jonas Nunes , Pinheiro Helena Saraiva Koenow , Bueno Mateus Marques , Junior Waldir De Carvalho , Pereira Nilson Rendeiro , Carvalho Daniel Costa de , Soares Paula Fernanda Chaves
Published: September 2020

The environmental resilience is strictly dependent of water availability. The identification of priority areas is important to conservation aid land-use planning and urban expansion, conservation, and policy strategy. The goal was to identify priority areas aiming water provision and environmental conservation at the “Canal do Rio Guandu” watershed in Rio de Janeiro, Brazil. To address the goal four micro-basins included in the watershed were selected to optimize collect field data and create the criteria to define the priority levels. Based on prior literature, legacy and field data, an assessment method was proposed based on map algebra with support of Geographical Information System, gathering professional tacit knowledge with spatial data to support the selection of strategic areas. The approach based on was successful to select primarily priority areas and can contribute to regulate the local policies, pointing out areas that can connect legally protected areas with forested fragments, which presents great importance to urban and rural supply.
Development of Fraud Prevention (FP) Model Using the Theory of Planned Behavior
Authors: Rosli Razif , Mohamed Intan Salwani , Mohamed Nafsiah , Othman Rohana , Rozzani Nabilah
Published: September 2020

Fraud is a global issue overwhelming all aspects of human economic and business activities as it poses potential threat of financial losses. Many detection and prevention mechanisms, such as internal control systems, operational audits, codes of conduct and forensic accounting have been applied in organizations aiming to effectively mitigate internal fraud activities. However, fraud is still occurring. This study is therefore aimed to develop a multidimensional theoretical model which explains factors (categorized under attitude, subjective norms and perceived behavioral controls) that influence Levers of Controls (LOC); how level of LOC could influence fraud prevention framework as well as whether LOC could mediate the relationship between planned behavioral variables and effective fraud prevention framework. Concerning more on the adoption and post-adoption stages regarding drivers of LOC, this study constructs a model in attempt to close the knowledge gaps found in prior studies. From literature review, theories relating with fraud prevention and behavioral controls were examined to identify knowledge gaps within previous measurements of effective fraud prevention framework. A multidimensional Fraud Prevention (FP) model is then developed by innovating the Theory of Planned Behavior (TPB).
Learning and Loving in Paradise Lost
Authors: Edwards Karen L.
Published: September 2020

The representation of learning in Paradise Lost provides an antidote to the pressured, anxiety-producing experience of higher education today. Demonic, divine, and prelapsarian ways of thinking in the poem prepare the ground for showing that Adam and Eve's way of learning is not a lesser process after the Fall but rather the sign of their having taken possession of their full humanity. Their postlapsarian errors and failures lead to the rethinking that learning depends upon, while the need to nurture their intimate, loving, and complex relationship ensures that their learning does not become abstruse and separate from their lived experience.
“Facile Gates:” Walls and Identity Across Paradise Lost and Along the US-Mexico Border
Authors: Bartlett Sydney
Published: September 2020

This article compares the respective functions of the US-Mexico border wall and borders within Paradise Lost. Supposed threats to economic, physical, and cultural property encourage modern nation-states to continue constructing territorial borders despite a growing body of work that contests the efficacy of walls in deterring unauthorized migration. More effective are the symbolic capacities of walls and their ability to articulate the people and cultures they enclose.
Milton's Timeless Music
Authors: Rosenblatt Jason P.
Published: September 2020

Most of us in the twenty-first century are assailed by information to a degree that could not have been predicted twenty-five years ago, and that comes at the price of an attenuated ability to respond to pure form. Music, the least representational of the arts, is the closest to pure form. And to the extent that poetry is itself a music, the aesthetic experience it offers makes recovery at least as pleasurable as discovery, just as a long-form musical composition may sound better if we have already heard it. Readers of Milton, supplying their own examples, can recall numerous passages whose music continues to give pleasure. This article provides one reader's experience of Milton's poetry, with different passages exerting their particular appeal in youth, middle age, and old age.
Structural Repair Strategies for LAMA2 MD using Linker Proteins
Authors: Ruegg Markus
Published: September 2020

LAMA2-related muscular dystrophy (LAMA2 MD or MDC1A) is caused by mutations in LAMA2, the gene encoding laminin-α2, the long arm of the heterotrimeric (α2, β1, γ1) protein laminin-211 (Lm-211). In most LAMA2 patients, biallelic mutations in LAMA2 result in the complete absence of Lm-211, the main laminin found in muscle basement membrane. As the cDNA encoding laminin-α2 is large (> 9kb), and the protein does not tolerate even subtle mutations because of its interaction with β1- and γ1-laminin chains, gene replacement or gene editing strategies are difficult to envisage. By studying the molecular mechanisms involved in the disease, we detect compensatory expression of laminin-α4, giving rise to Lm-411 (α4, β1, γ1) in LAMA2 patients’ biopsies and laminin-α2-deficient mice. Based on in vitro studies, Lm-411 was shown to be deficient in binding to muscle fibers and in forming a fully assembled basement membrane. In an attempt to restore muscle fiber binding and basement membrane assembly, we have designed two small, AAV-compatible linker proteins1. One linker, called mini-agrin (mag), works to mediate binding of Lm-411 to the muscle fiber via α-dystroglycan. The second linker, called αLNNd, involving the N-terminal part of laminin-α1 and the laminin-binding site of nidogen-1, allows polymerization of Lm-411. When expressed transgenically in dyW/dyW mice, a mouse model for LAMA2 MD, the two linkers ameliorate the muscle dystrophy, increase overall body weight and drastically extend lifespan. We will also report on our current efforts to evaluate the therapeutic potential of the linker proteins when expressed postnatally at different time points using AAV-mediated delivery to dyW/ dyW mice. These experiments aim to provide essential information for a possible translation of the linker strategy into LAMA2 MD patients.

1 Reinhard. S. Lin, K. McKee, S.Meinen, M. Sury, P. Yurchenco, M. Rüegg. Effect of linker protein expression on LAMA2-related muscular dystrophy (MDC1A). Neuromuscular Disorders (2019), 29, S164.
Cure CMD, a Patient-Centered Organization: Pivotal Role to Help Move Emerging Therapeutics into Clinical Practice for the Congenital Muscular Dystrophies
Authors: Dziewczapolski Gustavo
Published: September 2020

The mission of Cure CMD is to promote research directed to find a cure for the Congenital Muscular
Dystrophies (CMD). Cure CMD, a patient advocacy organization, was founded in 2008 by three parents
whose children were affected by understudied forms of CMDs. Just before the end of the 20th century,
the genes and coded proteins responsible for the clinicopathological presentations of CMD started to
be revealed. Merosin or laminin 211 was the first dysfunctional protein identified in western cases of
CMD. Later, a lack of another protein, collagen VI, was found to cause a different type of CMD. Today, it
is recognized that the five main subtypes of CMD involve proteins in four locations of the muscle cell:
the extracellular matrix (ECM) where laminin 211 and collagen VI reside (LAMA2-CMD and ColVI-CMD);
the ECM-sarcoplasm interface where the á-dystroglycan is the affected protein, either, as a consequence
of mutations in its coding gene (primary Dystroglycanopathy) or mutations in various glycosyltransferase
proteins (secondary Dystroglycanopathies); the sarcoplasmic reticulum, location of the SEPN1 protein
(Selenon or SEPN1-CMD); and the intranuclear membrane where the mutated protein lamin A/C
provokes the CMD subtype (LMNA or L-CMD).

The CMDs are a clinically and genetically heterogeneous group of NMDs. The symptoms are evident at
birth or before the second birthday. They present progressive weakness and hypotonia and share
characteristic biopsy findings. Several disease-related complications include nutritional deficiencies
due to feeding difficulties, joint contractures, and respiratory insufficiency. In some cases, heart
complications and cognitive impairment, are also present. Promising developments in biotechnology
and a deeper understanding of the underlying pathophysiological mechanisms responsible for the
different CMD types have facilitated design potential therapeutic schemes targeted at molecular, and
biochemical pathways. Through close collaboration with researchers, clinicians, patients, families,
dedicated volunteers, other foundations and donors, Cure CMD, has achieved significant impact in its
first decade as a nonprofit organization: launched two clinical trials; completed a five-year natural
history study with the NIH to characterize clinical trial endpoints; grew the Congenital Muscle Disease
International Registry (CMDIR) with approximately 3,000 registrants worldwide and, funded over $2
million in research grants. We will describe tools and programs Cure CMD has to offer to accelerate the
“bench-to-bedside” translation of therapies.
Congenital Muscular Dystrophy and Neuromuscular Disorders: An Overview
Authors: Abdel Aleem Alice
Published: September 2020

The muscle unit structure has been used to classify neuromuscular disorders (NMDs) according to the
site of involvement. The four main muscle unit components involve the: 1) Neuronal Anterior Horn Cell
(AHC). 2) Nerve Fiber (axon) transmitting the signal from the AHC to the skeletal muscle fibers. 3)
Neuromuscular junction (NMJ) (the cleft between nerve fiber’s endings and muscle sarcolemma
membrane). 4) Contractile unit (muscle belly that contains several muscle bundles; every bundle has
100-1000 muscle fibers). The myofibrils (muscle fibers) are the most complex component in terms of
the extensive number of proteins that code for the myofibers’ proteins and molecules. Spinal muscular
atrophy (SMA) presents the well-known AHC-related muscular diseases. The group of axons-related
NMDs (Neuropathy), called “Charcot Marie Tooth (CMT) diseases”, involves several subtypes and are of
different inheritance pattern. The NMJ-related NMDs is collectively named as “Myathenias”, of which are
the congenital myathenic syndromes. The muscle tissues (myofibrils) are associated with the most
extensive group of muscle diseases. Congenital muscular dystrophies (CMDs) and congenital
myopathies are primary muscle diseases that appear since birth and run progressive severe courses.
Neuromyology is currently a neurology sub-specialization that is highly effective in better clinical
identification of such significant genetically heterogeneous and clinically overlapping groups of NMDs
and CMDs. The advances in sequencing technologies have contributed towards uncovering the widerange
of genetic heterogeneity in NMDs. To date, over 600 genes were characterized in association with
congenital myopathies, dystrophies, and NMDs.

The future is holding truly promising cures for NMDs’ patients, through the emerging gene therapies, however its extremely high cost challenges the possibility for large-scale applications.
MRI Role in Imaging Myopathies
Authors: Mohammed Omar O.
Published: September 2020

Myopathies in pediatrics require comprehensive assessment of the affected child. One of these
assessments is related to muscles’ signal and atrophic changes which are easily detected by MRI
screening. This requires only a limited study of the whole body; hence, integration of a battery of
investigations including MRI imaging that could be very helpful with the diagnosis and follow up, as
well.
IMPACTO DO ENRIQUECIMENTO AMBIENTAL SOBRE O COMPORTAMENTO DE CÃES E DIGESTIBILIDADE DA DIETA EM CANIL EXPERIMENTAL
Authors: Ramos Tatiane Aparecida , Bill Kaelle Gislaine Cristina , Risolia Larissa Wunsche , Do Rosário Bárbara Cristina , De Oliveira Simone Gisele , Félix Ananda Portella
Published: September 2020

Cães de canis experimentais ficam alojados individualmente no período de experimentação, podendo causar assim, alguns problemas comportamentais, os quais prejudicam seu bem-estar. Com isso, uma ferramenta que pode ser utilizada para minimizar esses efeitos e melhorar o bem-estar dos animais é o enriquecimento ambiental.  Assim, o objetivo desse trabalho foi avaliar o comportamento de cães de canil experimental e a digestibilidade da dieta, com e sem enriquecimento ambiental. Foram utilizados oito cães adultos, os quais permaneceram 10 dias sem enriquecimento ambiental, seguidos por 10 dias com, totalizando 20 dias de experimento. O enriquecimento utilizado foi uma esfera oca, contendo furos, na qual o alimento era liberado conforme manipulação pelos cães. A dieta foi fornecida duas vezes ao dia. Foram realizados dois ensaios de digestibilidade, com a mesma dieta, sendo um no período sem enriquecimento e outro com. Cada ensaio de digestibilidade teve cinco dias de adaptação à dieta, seguido por cinco dias de coleta total de fezes. Foram observados os comportamentos dos cães durante o início e final de cada período. Houve aumento no tempo comendo (0,3% para 1,2%) e no comportamento exploratório (0,3% para 1,7%) dos cães no período que foi utilizado enriquecimento ambiental (P>0,05). Houve diminuição da coprofagia (1 vez para 0) no final do período que os animais estavam com enriquecimento (P<0,05). Os demais comportamentos não diferiram (P>0,05). Não houve diferença na digestibilidade da dieta mensurada sem e com enriquecimento (P>0,05). Com isso, o enriquecimento ambiental melhora alguns comportamentos, auxiliando no bem-estar de cães de canil experimental, sem interferir na mensuração da digestibilidade da dieta.
Investigation of the Relationship Between the Sports Status of Secondary School Students and Their Emotional Intelligence Levels
Authors: Çelebi Murat
Published: September 2020

The aim of this study is to investigate the sports status and emotional intelligence levels of secondary school students. 1027 secondary school students participated in the research. As a data collection tool in the research; personal information form, parent consent form and voluntary consent form were used. In measuring the emotional intelligence levels of students, a 60-item measurement tool developed by Bar-on, applicable to children and adolescents was used, and the scale was adapted to Turkish by Köksal (2007). Descriptive statistics, ANOVA and t-test techniques were used in the analysis of the data. As a result of the analyses, there was a statistically significant difference in interpersonal, adaptability and positive effect (p < 0.05) of the emotional intelligence sub-dimensions and the gender variable. When the emotional intelligence levels of the students were analyzed according to the variable of sports status, a significant difference was observed in the adaptability dimension and in the total score of emotional intelligence in favor of those who exercise sports (p < 0.05). When the emotional intelligence levels of the students were examined according to the class level variable, there were significant differences in the total score of emotional intelligence, stress management and adaptability (p < 0.05). Moreover, in the examination of the emotional intelligence levels of the students according to their mother’s education levels, a significant difference was found only in the adaptability dimension (p < 0.05). For their father’s education levels, there was a significant difference in the interpersonal and the adaptability dimensions (p < 0.05).
An Analysis of the Performance of European Union Countries in the Light of Europe 2020 Strategy Indicators
Authors: Grimaccia Elena
Published: September 2020

In this paper, an analysis of the Europe 2020 strategy indicators has been carried out. The strategy defined three priorities for European countries: Smart, Inclusive and Sustainable growth. These goals (developing an economy based on knowledge and innovation, fostering high employment levels, and promoting a more resource-efficient and greener economy) were measured by eight headline indicators, related to employment, research and innovation, renewables and energy, education and poverty. For each indicator, a target has been set, and the eight indicators are subject to regular statistical monitoring and reporting. Europe 2020 is perhaps not a complete set of indicators for measuring the progress of societies and the quality of life of their citizens, but it is a very important recognition of European institutions that GDP alone is not enough and that it must necessarily be integrated with measures that take into account equity and sustainability. The paper analyses the trends of the Europe 2020 indicators, considering the target reached or not, synthesizing the results using an Alkire-Foster method and clustering the 27 European countries, in order to highlight convergence processes among the Member States (MSs) in the ten years taken into account by the Strategy. After almost10 years, the EU has not reached most of the targets set in 2010, and many MSs are well behind schedule.
Ethics work for good participatory action research
Authors: Abma Tineke
Published: September 2020

Ethics work for good participatory action research: Engaging in a commitment to epistemic justice

Participatory and responsive approaches to research strive to be democratic, inclusive and impactful. Participatory researchers share a commitment to epistemic justice and actively engage citizens and users as well as other stakeholders in the co-creation of knowledge for social change. While more and more researchers and policymakers feel attracted to these approaches in practice, the normative ideals of social inclusion and justice are sometimes hard to realize, because of established interests, power relations and system requirements. In this article I argue that participatory researchers and evaluators have a moral responsibility to do ‘ethics work’. This is more than just following ethical principles and codes of conduct. ‘Ethics work’ entails the labour and effort one puts into recognizing ethically salient aspects of situations, developing oneself as a reflexive practitioner, paying attention to emotions and relationships, collaboratively working out the right course of action and reflecting in the company of critical friends. In this article I present the theory and ethics of participatory approaches, illustrate ethical issues and ethics work related to collaboration, politics and power, and share lessons based on ten years of practice in the field of health and social well-being.
Over de omvang van het hoger beroep
Authors: Rasterhoff M. (Menco) , Bektesevic D. (Dino)
Published: September 2020

Over de omvang van het hoger beroep

In hoger beroep bestaat regelmatig onduidelijkheid over de uitleg van de tenlastelegging en de vraag of sprake is van cumulatieve feiten. In deze bijdrage verkennen de auteurs het wettelijk systeem en de mogelijke belangen bij beperking van het hoger beroep. Vervolgens analyseren zij de rechtspraak van de gerechtshoven en doen zij enkele aanbevelingen.
Brand equity e valor de marca: proposição e validação de um modelo
Authors: Romanello Denis Lino , Freire Otavio , Quevedo-Silva Filipe , Santos Eduardo Biagi Almeida
Published: September 2020

Objetivo: Baseado na literatura de valor de marca e suas fontes de valor, o presente estudo propõe a ampliação do modelo de construção do patrimônio de marca, tendo como incremento o conceito de valor de marca e seus antecedentes.Método: Para testar o modelo, um survey foi operacionalizado junto a 330 consumidores de moda surfwear. A análise do modelo se deu por meio de modelagem de equações estruturais com uso da ferramenta PLS.Originalidade/Relevância: Propõe-se um modelo para análise da construção do valor e do patrimônio de marca.Resultados: Os resultados apontam a relevância do valor percebido para a construção do patrimônio de marca, sendo antecedida pelo conhecimento, associações, enquadramento, significado e qualidade percebida.Contribuições teóricas/metodológicas: O estudo buscou analisar as dimensões do patrimônio de marca e investigar as fontes de valor na sua operacionalização apontado dois caminhos relevantes a serem investigados. O primeiro caminho tem como ponto de partida a consciência de marca, que permite aos consumidores a formação de associações à marca. O segundo caminho proposto no modelo conceitual inicia-se pela percepção de qualidade.Implicações gerenciais: Os resultados indicam que os gestores de marca devem promover uma comunicação de marca abrangente e atraente, a fim de aumentar o conhecimento da marca e associações positivas, sempre buscando maximizar o valor da marca. O foco deve estar na criação de um alinhamento entre a imagem da marca e o perfil do consumidor, pois é importante que o consumidor se identifique com a imagem que a marca promove.
Irregularities in Customer Acquisition and in Advertising by Compensation Advisors
Authors: Dudziec-Rzeszowska Wioletta
Published: September 2020

This article presents selected examples of irregularities in customer acquisition and in advertising by compensation advisors. The former issue answers a question whether advertising restrictions apply to compensation advisors and if they are liable to disciplinary action in this respect, whereas the latter describes methods and ways of attracting clients. With regard to unethical methods of advertising used by the advisors, two acts of law have been referred to, namely: the Anti-Unfair Competition Act of 16 April 1993 and the Act on the Prevention of Unfair Market Practices of 23 August 2007, basing mainly on Polish case-law. The following research approaches have been applied: formal, statistical descriptive and systemic methodologies. Moreover, conclusions de lege ferenda have been presented.
Estrutura espacial e mobilidade urbana: estudo sistêmico aplicado à Região Metropolitana de Goiânia
Authors: Naciff Yordana Dias das Neves , Kneib Erika Cristine
Published: September 2020

A dificuldade de se compreender a organização das cidades instiga a busca por descobrir uma lógica na dinâmica urbana de maneira mais abrangente e não fragmentada. Trazer o pensamento sistêmico para o urbanismo, ou seja, assumir as cidades enquanto sistemas complexos, oferece uma possibilidade de abordagem integral do espaço urbano. Neste trabalho, considera-se que a estrutura espacial da cidade é um macrossistema repleto de campos a serem investigados, buscando a compreensão das relações entre esses campos e suas interações com a cidade. A mobilidade urbana, enquanto uma das áreas de análise, é considerada um campo essencial para o entendimento da estrutura socioeconômica e espacial intraurbana, conectando pessoas ao território urbano. Esse estudo adota como base a análise sistêmica aplicada à mobilidade dentro do contexto da estrutura espacial urbana. Objetiva-se, para tal, identificar e analisar as relações entre a estrutura espacial e a mobilidade urbana na Região Metropolitana de Goiânia (RMG). A pesquisa é de natureza básica, exploratória, descritiva e baseada principalmente em análises quali-quantitativas. Adota-se o uso da análise espacial georreferenciada a partir de um modelo inferencial que busca expor a relação entre eixos escolhidos. O modelo adotado é o de autocorrelação espacial, construído a partir de uma análise exploratória para identificação de localizações atípicas (outliers) e padrões de associação espacial (clusters) por meio de mapas das variáveis em estudo. Como resultado da pesquisa, explora-se as relações existentes entre a estrutura espacial e a mobilidade urbana na RMG testando a hipótese de a teoria sistêmica ser uma ferramenta capaz de auxiliar na compreensão de relações urbanas.