bluuebunny/crossref_metadata_2025_split huggingface dataset:
Axonopathy in the Central Nervous System Is the Hallmark of Mice with a Novel Intragenic Null Mutation of Dystonin
Authors: Seehusen Frauke , Kiel Kirsten , Jottini Stefano , Wohlsein Peter , Habierski Andre , Seibel Katharina , Vogel Tanja , Urlaub Henning , Kollmar Martin , Baumgärtner Wolfgang , Teichmann Ulrike 
Published: July 2016

Abstract
Dystonia musculorum is a neurodegenerative disorder caused by a mutation in the dystonin gene. It has been described in mice and humans where it is called hereditary sensory autonomic neuropathy. Mutated mice show severe movement disorders and die at the age of 3–4 weeks. This study describes the discovery and molecular, clinical, as well as pathological characterization of a new spontaneously occurring mutation in the dystonin gene in C57BL/6N mice. The mutation represents a 40-kb intragenic deletion allele of the dystonin gene on chromosome 1 with exactly defined deletion borders. It was demonstrated by Western blot, mass spectrometry, and immunohistology that mice with a homozygous mutation were entirely devoid of the dystonin protein. Pathomorphological lesions were restricted to the brain stem and spinal cord and consisted of swollen, argyrophilic axons and dilated myelin sheaths in the white matter and, less frequently, total chromatolysis of neurons in the gray matter. Axonal damage was detected by amyloid precursor protein and nonphosphorylated neurofilament immunohistology. Axonopathy in the central nervous system (CNS) represents the hallmark of this disease. Mice with the dystonin mutation also showed suppurative inflammation in the respiratory tract, presumably due to brain stem lesion-associated food aspiration, whereas skeletal muscles showed no pathomorphological changes. This study describes a novel mutation in the dystonin gene in mice leading to axonopathy in the CNS. In further studies, this model may provide new insights into the pathogenesis of neurodegenerative diseases and may elucidate the complex interactions of dystonin with various other cellular proteins especially in the CNS.
Genomics of Natural Populations: How Differentially Expressed Genes Shape the Evolution of Chromosomal Inversions inDrosophila pseudoobscura
Authors: Fuller Zachary L , Haynes Gwilym D , Richards Stephen , Schaeffer Stephen W 
Published: July 2016

AbstractChromosomal rearrangements can shape the structure of genetic variation in the genome directly through alteration of genes at breakpoints or indirectly by holding combinations of genetic variants together due to reduced recombination. The third chromosome of Drosophila pseudoobscura is a model system to test hypotheses about how rearrangements are established in populations because its third chromosome is polymorphic for &gt;30 gene arrangements that were generated by a series of overlapping inversion mutations. Circumstantial evidence has suggested that these gene arrangements are selected. Despite the expected homogenizing effects of extensive gene flow, the frequencies of arrangements form gradients or clines in nature, which have been stable since the system was first described &gt;80 years ago. Furthermore, multiple arrangements exist at appreciable frequencies across several ecological niches providing the opportunity for heterokaryotypes to form. In this study, we tested whether genes are differentially expressed among chromosome arrangements in first instar larvae, adult females and males. In addition, we asked whether transcriptional patterns in heterokaryotypes are dominant, semidominant, overdominant, or underdominant. We find evidence for a significant abundance of differentially expressed genes across the inverted regions of the third chromosome, including an enrichment of genes involved in sensory perception for males. We find the majority of loci show additivity in heterokaryotypes. Our results suggest that multiple genes have expression differences among arrangements that were either captured by the original inversion mutation or accumulated after it reached polymorphic frequencies, providing a potential source of genetic variation for selection to act upon. These data suggest that the inversions are favored because of their indirect effect of recombination suppression that has held different combinations of differentially expressed genes together in the various gene arrangement backgrounds.
Thumb-Sucking, Nail-Biting, and Atopic Sensitization, Asthma, and Hay Fever
Authors: Lynch Stephanie J. , Sears Malcolm R. , Hancox Robert J. 
Published: July 2016

BACKGROUND:
The hygiene hypothesis suggests that early-life exposure to microbial organisms reduces the risk of developing allergies. Thumb-sucking and nail-biting are common childhood habits that may increase microbial exposures. We tested the hypothesis that children who suck their thumbs or bite their nails have a lower risk of developing atopy, asthma, and hay fever in a population-based birth cohort followed to adulthood.

METHODS:
Parents reported children’s thumb-sucking and nail-biting habits when their children were ages 5, 7, 9, and 11 years. Atopic sensitization was defined as a positive skin-prick test (≥2-mm weal) to ≥1 common allergen at 13 and 32 years. Associations between thumb-sucking and nail-biting in childhood, and atopic sensitization, asthma, and hay fever at these ages were assessed by using logistic regression with adjustments for sex and other potential confounding factors: parental atopy, breastfeeding, pet ownership, household crowding, socioeconomic status, and parental smoking.

RESULTS:
Thirty-one percent of children were frequent thumb-suckers or nail-biters at ≥1 of the ages. These children had a lower risk of atopic sensitization at age 13 years (odds ratio 0.67, 95% confidence interval 0.48–0.92, P = .013) and age 32 years (odds ratio 0.61, 95% confidence interval 0.46–0.81, P = .001). These associations persisted when adjusted for multiple confounding factors. Children who had both habits had a lower risk of atopic sensitization than those who had only 1. No associations were found for nail-biting, thumb-sucking, and asthma or hay fever at either age.

CONCLUSIONS:
Children who suck their thumbs or bite their nails are less likely to have atopic sensitization in childhood and adulthood.
DEPENDENCE OF AIR SPRING PARAMETERS ON THROTTLE RESISTANCE
Authors: Reidemeister O. H. , Kivisheva A. V. 
Published: July 2016

Purpose. In this paper it is necessary to conduct: 1) research and analyse the influence of throttle element pneumatic resistance on elastic and damping parameters of air spring; 2) to obtain the dependence of air spring parameters on throttle element pneumatic resistance value. Methodology. The work presents the elaborated model of the air spring as a dynamic system with three phase coordinates (cylinder pressure, auxiliary reservoir pressure, cylinder air mass). Stiffness and viscosity coefficients were determined on the basis of system response to harmonic kinematic disturbance. The data for the analysis are obtained by changing the capacity of the connecting element and the law of pressure variation between the reservoir and the cylinder. The viscosity coefficient is regarded as the viscosity ratio of the hydraulic damper, which for one oscillation cycle consumes the same energy as the air spring. The process of air condition change inside the cylinder (reservoir) is considered to be adiabatic; the mass air flow through the connecting element depends on the pressure difference. Findings. We obtained the curves for spring viscosity and stiffness coefficients dependence on the throttle resistance at three different laws, linking airflow through the cylinder with the pressure difference in cylinder and reservoir. At both maximum and minimum limiting resistance values the spring viscosity tends to zero, reaching its peak in the mean resistance values. Stiffness increases monotonically with increasing resistance, tends to the limit corresponding to the absence of an auxiliary reservoir (at high resistance) and the increase in cylinder volume by the reservoir volume (at low resistance). Originality.The designed scheme allows determining the optimal parameters of elastic and damping properties of the pneumatic system as function of the throttle element air resistance. Practical value.The ability to predict the parameters of elastic and damping properties of the pneumatic system as function of the throttle element air resistance will improve the running performance of carriages, the comfort of passenger transportation and reduce the wear of the rolling stock and the track caused by interaction of carriage and rails.
A Randomized Phase II Study of FOLFOX With or Without the MET Inhibitor Onartuzumab in Advanced Adenocarcinoma of the Stomach and Gastroesophageal Junction
Authors: Shah Manish A. , Cho Jae-Yong , Tan Iain B. , Tebbutt Niall C. , Yen Chia-Jui , Kang Alice , Shames David S. , Bu Lilian , Kang Yoon-Koo 
Published: July 2016

Abstract

Background.
The phase II YO28252 study (01590719) examined first-line onartuzumab plus mFOLFOX6 in patients with metastatic, human epidermal growth factor receptor 2-negative adenocarcinoma of the stomach or gastroesophageal junction. MET immunohistochemistry expression as a biomarker of onartuzumab activity was also examined.

Patients and Methods.
Patients were randomized 1:1 to receive standard mFOLFOX6 plus onartuzumab (10 mg/kg) or placebo in 2-week cycles for 12 cycles, followed by onartuzumab or placebo until disease progression. Coprimary endpoints were progression-free survival (PFS) in intent-to-treat (ITT) and MET-positive populations. The target hazard ratio (HR) was 0.70 for patients in the ITT group and 0.60 in the MET-positive population. Secondary endpoints were overall survival (OS), overall response rate (ORR), and safety.

Results.
Overall, 123 patients were enrolled (n = 62 onartuzumab, n = 61 placebo). Median PFS was 6.77 versus 6.97 months for onartuzumab versus placebo, respectively (HR, 1.08; 95% confidence interval [CI], 0.71–1.63; p = .71). In the MET-positive population, median PFS was 5.95 versus 6.80 months, onartuzumab versus placebo (HR, 1.38; 95% CI, 0.60–3.20; p = .45). Median OS was 10.61 months for onartuzumab versus 11.27 months for placebo) (HR, 1.06, 0.64–1.75; p = .83). In the MET-positive population, median OS was 8.51 versus 8.48 months for onartuzumab versus placebo, respectively (HR, 1.12, 95% CI, 0.45–2.78; p = .80). ORR was 60.5% for the onartuzumab group and 57.1% for placebo. Grade 3–5 adverse events (AEs) were seen in 88.3% of patients receiving onartuzumab and in 78.3% of patients receiving placebo, with serious AEs in 55% and 40%, respectively.

Conclusion.
The addition of onartuzumab to mFOLFOX6 in gastric cancer did not improve efficacy in an unselected population or in a MET immunohistochemistry-positive population.
On multivariable cumulant polynomial sequences with applications
Authors: Di Nardo Elvira 
Published: July 2016

A new family of polynomials, called cumulant polynomial sequence, and its extension to the multivariate case is introduced relied on a purely symbolic combinatorial method. The coefficients are cumulants, but depending on what is plugged in the indeterminates, moment se- quences can be recovered as well. The main tool is a formal generalization of random sums, when a not necessarily integer-valued multivariate random index is considered. Applications are given within parameter estimations, L\'evy processes and random matrices and, more generally, problems involving multivariate functions. The connection between exponential models and multivariable Sheffer polynomial sequences offers a different viewpoint in employing the method. Some open problems end the paper.
Getting Back to the Sandbox: Designing a legal policy clinic
Authors: Patton William Wesley 
Published: July 2016

<p>This article will analyze contemporary educational psychology in an attempt to: (1) determine whether a sandbox can and/or should be added to the law school curriculum; (2) describe a constructivist learning environment with the goal of providing law students self-selected pro bono publico projects that may help internalize a life-long goal of public service; and, provide an interdisciplinary model that is feasible both in the large university law schools and in small and/or free-standing law schools. The second half of the article will describe my attempts to build a sandbox model into my Legal Policy Clinic.</p>
How to Discover Students’ Talents and Turn Them into Teaching
Authors: Urban Michal 
Published: July 2016

<p>Some law students were born to teach. Their professors’ job is to help them to discover their talents and turn them into teaching. This paper offers a description of the Prague Street Law Programme with an emphasis on the way of working with law students, who possess clear talent for teaching. Students enrolling for Street Law clinics are students of law, who have obviously decided to study law rather than pedagogy. However, when being provided with an opportunity to teach, some law students prove to possess clear pedagogical talents, which they themselves might not realise. This article analyses Czech experience regarding running Street Law clinics, whose aim goes beyond traditional aims of Street Law clinics, i.e. beyond putting theory into practice, looking at law through the eyes of the lay people, communicating legal matters to lay people, improving presentation skills and serving the community. In addition to these highly beneficial traditional aims of Street Law clinics, Prague Street Law programme also seeks to create an environment, in which law students might realise their pedagogical talents, equip them with necessary methodology, find enjoyment in teaching and efficiently turn their talents into the teaching of law. The paper focuses in particular on the description of the way Prague Street Law programme is designed to support the described goals and lists a number of concrete teaching methods, which have been employed to stimulate students’ pedagogical development, since these might serve as a source of inspiration for other clinicians.</p>
ABA/CEELI’s clinical legal education programme in Serbia
Authors: Stankovic Karajovic Emilija 
Published: July 2016

<p>The goal of the CEELI Legal Education Reform Program in Serbia has been to assist Serbian law faculties in reforming the curriculum so that law students become lawyers who can contribute to the development of the rule of law and the transition to a market economy. As a country in transition, Serbia must prepare future lawyers who are capable of absorbing and implementing the breadth of changes underway in the legal system. Unfortunately, in both its pedagogical methodology and its resources, the education predominantly provided to law students in Serbia is woefully inadequate. Education is typically based on memorisation of code provisions, with little opportunity for practice-based learning or creative thinking, and many of the textbooks used by law students date back to the socialist era.</p>
Histórias ‘em suspenso’: os Tembé ‘de Santa Maria’, estratégias de enfrentamento do etnocídio ‘cordial’
Authors: Beltrão Jane Felipe 
Published: July 2016

Os Tembé, ditos ‘de Santa Maria’, até recentemente não eram reconhecidos como Tenetehara, situação que, de certa forma,ainda perdura. Por mais de 100 anos suashistórias permaneceram ‘em suspenso’,como uma das faces do etnocídio ‘cordial’.Há pouco mais de 10 anos, eles começarama ouvir e a aprender narrativas produzidas por terceiros sobre si e, na luta por vencer a invisibilidade, passam a se afirmar Tembé pagando o alto preço da‘resistência’. Discutem-se a partir dos interlocutores(1) as histórias ‘herdadas’(produzidas por terceiros para auxiliar a luta); (2) as narrativas indígenas sobre os trajetos feitos até Santa Maria; e (3) a apropriação que os Tembé fazem da ‘herança’e das narrativas produzindo os fios da ‘resistência’ identitária, até mesmo requerendo que antropólogos produzam a sua história, como se eles não pudessem apresentar sua versão. Teríamos aí novas formas de fazer História?
Pedagogias da saudade: a formação histórica de consciências e sensibilidades saudosistas. A vida e o trabalho do poeta e professor português António Corrêa d’Oliveira
Authors: Muniz de Albuquerque Junior Durval 
Published: July 2016

Baseado no fato de que o aprendizado da história, de que o conhecimento de versões sobre o passado não se dá apenas no interior da instituição escolar, que outras pedagogias atuam na sociedade e se utilizam de versões do passado para a formação das consciências e sensibilidades de homens e mulheres, o texto parte da trajetória de vida do poeta e professor português António Corrêa d’Oliveira e se interroga sobre os usos que fez da história não apenas em sua obra, mas em sua vida. O texto tenta mostrar, acima de tudo, como a própria vivência da história, as próprias experiências sociais, culturais e temporais que sofrem os sujeitos conformam dadas maneiras de pensar e lidar com o histórico. Neste caso, mostramos a relação intrínseca entre uma consciência e uma sensibilidade saudosista e dadas maneiras de ensinar e de aprender a história.
Estado, editoras e ensino: o papel da política na produção, avaliação e distribuição dos livros didáticos de História no Brasil (1938-2012)
Authors: Silveira Matos Júlia , Kivanski de Senna Adriana 
Published: July 2016

A ampla distribuição de livros didáticos no Brasil, tanto no ensino Fundamental quanto no Médio, é garantida por uma política governamental de educação que atualmente se efetiva pelo Programa Nacional do Livro Didático (PNLD). Entretanto, a trajetória das políticas voltadas para a avaliação, regulação e distribuição dos livros didáticos no Brasil foi marcada por três momentos específicos da história nacional. O primeiro ainda no século XIX, com a criação da Comissão de Instrução Pública; o segundo, com a Comissão Nacional do Livro Infantil (1936), a Comissão Nacional do Ensino Primário (1938) e a Comissão Nacional do Livro Didático (1938); por fim, o terceiro momento tem a criação em 1985, já no final do governo militar, do Programa Nacional do Livro Didático (PNLD). No presente texto analisaremos o papel histórico desses órgãos no processo de seleção e distribuição dos livros didáticos para as escolas públicas no Brasil.
Ação Educativa a Distância: relato de uma prática com professores no Arquivo Público do Estado de São Paulo
Authors: Cristina Oliver Barbosa Andresa , Pereira de Freitas Carla Janaína , Plácido da Rosa Silva Stanley 
Published: July 2016

O artigo tem como objetivo analisar as ações direcionadas aos professores de ciências humanas dos ensinos fundamental e médio a partir do curso “O(s) uso(s) de documentos de Arquivo na sala de aula” na modalidade EAD, oferecido pelo Núcleo de Ação Educativa do Arquivo Público do Estado de São Paulo (Apesp). Entendendo-o como uma ação pioneira em arquivos públicos do país, discutiremos inicialmente como concebemos tais instituições, bem como os pressupostos teóricos e metodológicos que embasam o curso. Após uma reflexão sobre o papel dos arquivos na sociedade e suas possíveis contribuições para a educação, analisaremos o primeiro curso em EAD oferecido ao público externo, no segundo semestre de 2013.
Faça aqui o seu login: os historiadores, os computadores e as redes sociais online
Authors: Leal Pastor de Carvalho Bruno 
Published: July 2016

Há alguns anos, os computadores e, mais recentemente, as redes sociais onlineemergiram como um fenômeno social, político e cultural extremamente importante de nosso tempo, com reflexos nas mais diversas atividades profissionais e intelectuais. O artigo tem o objetivo de pensar a relação entre o historiador e os computadores, o historiador e as redes sociais na internet, em especial o caso da rede social Café História. Espera-se, assim, compreender melhor os pontos de contato das novas tecnologias da comunicação com a história.
A memória como patrimônio ou a História como prática social? Reflexões sobre práticas de memória e ensino de história na Pedagogia do Movimento Sem Terra
Authors: Lucini Marizete 
Published: July 2016

O artigo discute a memória produzida na prática social como patrimônio de homens e mulheres na luta pela terra. A reflexão que propomos funda-se na fenomenologia da Memória proposta por Paul Ricoeur, evidenciando-se que a História como uma prática social desenvolvida em um Assentamento de Reforma Agrária atua na produção de uma Memória que ao ser apropriada por esse grupo constitui seu patrimônio material e imaterial.
O amor no museu: uma experiência de ensino de História com objetos do amor romântico
Authors: Sousa Rios Kênia 
Published: July 2016

O artigo tem a intenção de compartilhar uma experiência de aula de história da Universidade Federal do Ceará, analisada com base na temática do amor romântico. O assunto colocou a história dos sentimentos como possibilidade de reflexão historiográfica e ao mesmo tempo ajudou a construir uma opção didática para o ensino de história entre alunos da graduação e alunos do ensino médio na forma de uma exposição no Museu Histórico do Ceará, em Fortaleza.
Experiências curriculares e formação de professores: uma tessitura coletiva no âmbito do Pibid/UFES
Authors: Pinto Junior Arnaldo , Azevedo Katia Valeska , Cardoso de Lima Mônica 
Published: July 2016

O artigo apresenta relatos de experiências vivenciadas no âmbito do Pibid/UFES, a partir dos quais os professores responsáveis refletem acerca dos desdobramentos na formação dos bolsistas de iniciação à docência e das próprias práticas de ensino e pesquisa. Desenvolvido em parceria com instituições públicas da cidade de Vitória (ES), o subprojeto de Licenciatura em História trabalhou com turmas de Ensino Fundamental e Médio para que os licenciados pudessem conhecer e debater distintas condições e demandas. Ao considerarmos as atuais dinâmicas escolares e perspectivas dos currículos acadêmicos, destacamos experiências que potencializam análises relativas aos projetos de ensino interdisciplinares, suas conexões com o perfil bacharelesco dos cursos de Licenciatura e a compreensão do que é conhecimento histórico para graduandos e estudantes da Educação Básica.
Stela Maria Meneghel
Authors: Robl Fabiane , Eduardo Dias de Mello Paulo , Sapag Ricci Claudia , Marinho-Araújo Claisy 
Published: July 2016

A entrevista discute o processo de Avaliação da Educação Superior no Brasil, sobretudo a história da criação da Comissão Especial de Avaliação (CEA) e da Comissão Nacional de Avaliação da Educação Superior (Conaes), além de problematizar a formulação das políticas de avaliação. Stela Maria Meneghelé Coordenadora Geral de Controle de Qualidade da Educação Superior da Diretoria de Avaliação da Educação Superior (Daes) do Instituto Nacional de Estudos e Pesquisas Educacionais Anísio Teixeira (Inep). Bacharel e licenciada em Letras (1987), mestre em Educação (1994) e doutora (2001) pela Universidade Estadual de Campinas (Unicamp), com pós-doutorado no Instituto Internacional para a Educação Superior na América Latina e Caribe (Iesalc/Unesco, 2008), atuou como professora titular da Universidade Regional de Blumenau (2001-2009), no Programa de Pós-Graduação em Educação. Colaborou na elaboração (2003) e na implantação (2004-2006) do Sistema Nacional de Avaliação de Educação Superior (Sinaes), ao atuar como Secre tária-Executiva e Assessora Técnica da Comissão Nacional de Avaliação da Educação Superior (Conaes/MEC). Integrou a Comissão de Implantação da Universidade da Integração Latino--Americana (Unila, 2008-2010), tendo coordenado o primeiro curso oferecido por essa universidade: o Curso Latino--Americano de Especialização em Políticas Públicas e Avaliação da Educação Superior (CLAEPPAES). Em 2009 e 2010 atuou como professora visitante na Universidade Federal do Paraná (UFPR), instituição tutora da Unila. Em agosto de 2010, assumiu Consultoria Unesco para Implantação da Pró-Reitoria de Pesquisa, Pós-Graduação e Extensão da Universidade de Integração Internacional Luso Afro--Brasileira (Unilab), onde foi pró-reitora (2011-2012).