bluuebunny/crossref_metadata_2025_split huggingface dataset:
Milton's Timeless Music
Authors: Rosenblatt Jason P. 
Published: September 2020

ABSTRACT
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.
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.
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.
Da luta “pela” terra a luta “na” terra: a trajetória dos assentados de Zumbi dos Palmares, Mato Grosso
Authors: Miorin Vera Maria Favila , Oliveira Iolanda Lopes de , Sturza José Adolfo Iiriam 
Published: September 2020

O rural brasileiro sempre foi marcado por um histórico de desigualdades sociais, revelando séculos de favorecimento à formação de latifúndios. Esse cenário propiciou um crescente movimento de lutas pela terra em todo país. Diante desta realidade o presente trabalho tem por objetivo discutir esse processo de luta pela terra e de luta na terra através da análise do Assentamento Zumbi dos Palmares no estado de Mato Grosso. O assentamento localizado no município de Dom Aquino, região sudeste do estado, foi fixado em um local em que as condições não permitem maior desenvolvimento, dificultando a reprodução social e econômica dos lotes familiares e consequentemente a permanência das famílias no assentamento. Através de uma metodologia baseada na observação participante e na utilização de questionarios e entrevistas foi possivel compreender melhor como todo esse movimento se deu em suas particularidades. Caracteristicas que por vezes acabam se revelando parte de um cenario nacional ao se analisar os assentamentos rurais de nosso país
Relationship between Economic Growth, Unemployment, Inflation and Current Account Balance: Theory and Case of Turkey
Authors: Dayıoğlu Tuğba , Aydın Yılmaz 
Published: September 2020

The relations between economic growth, unemployment, inflation and current account balance are analyzed theoretically and different comments on theoretical approaches are discussed in the study. Accordingly, while the unemployment-inflation relationship is considered with Phillips analysis and the scope of the growth-unemployment with Okun Law, the interaction between the current account balance and growth is shown with the equality of national income accounting. After the theoretical approaches described in detail with shared data and interpreted for Turkey. This study also examines the relation between the unemployment, inflation, economic growth, current account deficit with symmetric and asymmetric reserved causality tests were examined for the 2000Q1 − 2020Q4 period. The asymmetric hidden causality relationships between the series were researched with Hatemi-J (2012) method based on Toda-Yamamoto (1995) test in this study. When the relationship between the growth rate and the unemployment rate are examined between these years in Turkey it is observed that there is an inverse relationship between growth and unemployment, especially during crisis periods. After that to find this relationship we used symmetric and asymmetric causality. As a result of the estimates growth also has a one-way symmetrical causality relationship from negative shocks to negative inflation shocks. When the relationship between them is viewed only with one-way or two-way causality, there may be no relationship so the causality must be checked asymmetrically even to catch the assumption of the Okun’s law correctly for Turkey.
Lomber Disk Hernisi Cerrahisinde Uygulanan Farklı Anestezi Yöntemlerinin Karşılaştırılması
Authors: DOĞAN Erdal 
Published: September 2020

Giriş: Lomber disk hernisi cerrahisi beyin cerrahisi pratiğinde sık yapılan ameliyatlardan biri olup sıklıkla genel anestezi altında yapılmaktadır. Genel anesteziye alternatif yöntem olan spinal anestezi, tüm spinal cerrahi işlemlerinde uygulanabildiği gibi pek çok avantaja sahiptir. Kliniğimizde lomber disk hernisi cerrahisinde uygulanan farklı anestezi yöntemlerinin etkinliklerini karşılaştırmayı amaçladık.
Yöntemler: Bu çalışma hastane yönetimi onayı alındıktan sonra anesteziyoloji ve reanimasyon kliniğinde yapılmıştır. Kliniğimizde Mart 2017–Temmuz 2019 tarihleri arasında lomber disk hernisi nedeni ile spinal ya da genel anestezi altında elektif olarak opere edilen, ASA I-III, 18-70 yaş arası 234 hasta dosyaları retrospektif olarak incelendi. Hastaların demografik verileri, ameliyat süresileri, ilk mobilizasyon zamanları, efedrin yapılan hasta sayısı, postoperatif deksketorofen tüketimleri, postoperatif tramadol tüketimleri, herniasyon seviyeleri, spinal anestezi blok başarı oranı ve post spinal baş ağrısı gelişen hasta sayısı, hemodinamik parametreleri, operasyon anında ya da sonrasında gelişen komplikasyonlar kaydedildi.
Bulgular: Toplamda 234 hasta çalışmaya dahil edildi. Bu hastalardan 212 tanesi spinal anestezi, 22 tanesi genel anestezi altında ameliyat oldu. 3 hastada post spinal başağrısı gelişti. Postoperatif ilk analjezik ihtiyacı zamanı, total deksketorofen tüketimi, total tramadol tüketimi ve ameliyat süresi spinal anestezi grubunda anlamlı bir şekilde daha düşük bulundu.
Sonuç: Lomber disk cerrahisinde spinal anestezi etkili, güvenli ve iyi bir postoperatif ağrı konforu sağlamakla birlikte, ameliyatın uzun sürmemesinin beklendiği tek seviye herniasyonlarda daha yaygın bir şekilde kullanılabileceği düşüncesindeyiz.
Book of the Wars of the Lord (Num. 21:14–20)
Authors: Steiner Richard C. 
Published: September 2020

Num. 21:14 contains one of the most enigmatic phrases in the Pentateuch בּסוּ ָפה The usual interpretations of ֶאת turn the phrase into gibberish because they require the presence of a verb, which is nowhere to be found. Some scholars have supplied an understood verb; others have resorted to emendation. A better solution is available: ֶאת in our passage is a verb masquerading as a preposition. It is easily construed, without the slightest change, as an archaic apocopated/biliteral imperative of א-ת-י, meaning “come!”

This construal of ֶאת transforms our understanding of Num. 21:14–20 in many ways. For example, it reveals that ַעל־ ֵכּן יֵָא ַמר ְבּ ֵסֶפר ִמ ְלֲחמ ֹת הʹ ֶאת־ָו ֵהב , “On account of that, it is said in the Book of the Wars of the Lord, ‘Come to (Mount) Waheb’,” bears a striking resemblance to ַעל־ ֵכּן י ֹא ְמרוּ ַהמּ ֹ ְשׁ ִלים בּ ֹאוּ ֶח ְשׁבּוֹן , “On account of that, the bards say, ‘Come to Heshbon’,” thirteen verses later. More generally, the new construal, taken together with some pertinent geographic, ethnographic, and hydrologic facts, makes possible a new, more coherent reading of the entire passage.
Accuracy Implementation of Medical Record Management Information System with Waterfall Design System and ISO 9126
Authors: Widiyanto Wahyu Wijaya , Wulandari Sri 
Published: September 2020

Aims: Based on the observations of researchers, some health facilities still use manual processes / have not been documented by the information system resulting in slow service, this study aims to improve health services with a medical record information system.
Methodology: The method used in this study is an analysis of information systems with the waterfall method and accuracy testing with ISO 9126.
Results: The results of this medical record management information system run well based on black-box testing and white box results obtained both from an average value of 82 based on the ISO 9126 scale conversion table.
Conclusion: Based on the results of the average value obtained from the validation test carried out on 3 expert examiners, it can be concluded that the application for the validation system for the validation and distribution of this letter has met the ISO 9126 standard with an average good interpretation of a total value of 82, and according to be able to simplify the process of Patient Medical Record Data Management without neglecting the safety aspects of the validation and distribution process, minimizing data loss, simplifying the reporting process and facilitating the processing of patient medical record data.
Assessing Taro (Colocasia esculenta (L.) Schott) Leaf Blight Incidence, Severity, and Farmers’ Knowledge of the Disease in Fako Division of Cameroon
Authors: Takor Mandah Cicelia , Monono Ekwa Yawa , Ntane Ojong Agbor , Ngale Jemimah Evenye , Fontem Lum A. 
Published: September 2020

Taro leaf blight (TLB), a major disease of taro plant caused by Phytophthora colocasiae, a pseudo-fungus, that occurs in many taro producing areas, especially in the South West of Cameroon. A Survey was conducted to assess farmers’ knowledge, as well as to determine the incidence and the severity of the disease in taro fields. Structured questionnaires were administered in selected locations (Bokova, Ekona, Muea, Mile 16, Mutengene, and Likomba) of taro production. Two farms were selected with two quadrats of 64 m2 each were carved to assess disease incidence and severity on taro. The number of plants infected with taro leaf blight were counted and the area of the affected leaves measured was used to evaluate the disease incidence and disease severity. Data collected were subjected to ANOVA for complete randomized design and the means were compared using Tukey test at 0.05 probability levels. The results revealed that most of the farmers could identify the disease while practicing cultural methods in controlling the disease. The results also revealed significant differences (p = 0.001) in disease incidence and disease severity in all the localities, with Mile 16 showing the highest percentage mean of 96.53% and 85.59%, respectively. The severity scored showed high infection range of 3 – 3.95 in all locations except in Likomba (2.60). It could be concluded from the results that there were high prevalence of taro leaf blight disease in Fako Division. Good management strategies are therefore required to control Taro leaf blight and improve taro production in the area.