bluuebunny/crossref_metadata_2025_split huggingface dataset:
mTOR Hyperactivity Levels Influence the Severity of Epilepsy and Associated Neuropathology in an Experimental Model of Tuberous Sclerosis Complex and Focal Cortical Dysplasia
Authors: Nguyen Lena H. , Mahadeo Travorn , Bordey Angélique 
Published: January 2019

Tuberous sclerosis complex (TSC) and focal cortical dysplasia (FCD) are focal malformations of cortical development (FMCDs) that are highly associated with intractable epilepsy. TSC and FCD are mTORopathies caused by a spectrum of pathogenic variants in the mechanistic target of rapamycin (mTOR) pathway genes leading to differential activation of mTOR signaling. However, whether the degree of mTOR hyperactivity influences disease severity remains unclear. Here, we examined the effects of differential mTOR hyperactivity levels on epilepsy and associated neuropathology in a mouse model of TSC and FCD. Constitutively active Rheb (RhebCA), the canonical activator of mTOR complex 1 (mTORC1), was expressed in mouse embryos of either sex viain uteroelectroporation at low, intermediate, and high concentrations to induce different mTORC1 activity levels in developing cortical neurons. We found that RhebCAexpression induced mTORC1 hyperactivation and increased neuronal soma size and misplacement in a dose-dependent manner. No seizures were detected in the low RhebCAmice, whereas the intermediate and high RhebCAmice displayed spontaneous, recurrent seizures that significantly increased with higher RhebCAconcentrations. Seizures were associated with a global increase in microglial activation that was notably higher in the regions containing RhebCA-expressing neurons. These data demonstrate that neuronal mTOR hyperactivity levels influence the severity of epilepsy and associated neuropathology in experimental TSC and FCD. Overall, these findings highlight the importance of evaluating the outcome of individual variants on mTOR activity levels and support personalized medicine strategies based on patient variants and mTOR activity level for TSC, FCD, and potentially other mTORopathies.SIGNIFICANCE STATEMENTTuberous sclerosis complex (TSC) and focal cortical dysplasia (FCD) are epileptogenic cortical malformations caused by pathogenic variants in mechanistic target of rapamycin (mTOR) pathway genes leading to differential mTOR hyperactivation. Here, we present novel findings that neuronal mTOR hyperactivity levels correlate with the severity of epilepsy and associated neuropathology in a mouse model of TSC and FCD. Our findings suggest the need to evaluate the outcome of individual variants on mTOR activity levels in clinical assessments and support personalized medicine strategies based on patient variants and mTOR activity level. Additionally, we present useful modifications to a previously described mouse model of TSC and FCD that allows for titration of seizure frequency and generation of a mild to severe epilepsy phenotype as applicable for preclinical drug testing and mechanistic studies.
Arginine to Glutamine Variant in Olfactomedin Like 3 (OLFML3) Is a Candidate for Severe Goniodysgenesis and Glaucoma in the Border Collie Dog Breed
Authors: Pugh Carys A , Farrell Lindsay L , Carlisle Ailsa J , Bush Stephen J , Ewing Adam , Trejo-Reveles Violeta , Matika Oswald , de Kloet Arne , Walsh Caitlin , Bishop Stephen C , Prendergast James G D , Rainger Joe , Schoenebeck Jeffrey J , Summers Kim M 
Published: January 2019

AbstractGoniodysgenesis is a developmental abnormality of the anterior chamber of the eye. It is generally considered to be congenital in dogs (Canis lupus familiaris), and has been associated with glaucoma and blindness. Goniodysgenesis and early-onset glaucoma initially emerged in Border Collies in Australia in the late 1990s and have subsequently been found in this breed in Europe and the USA. The objective of the present study was to determine the genetic basis of goniodysgenesis in Border Collies. Clinical diagnosis was based on results of examinations by veterinary ophthalmologists of affected and unaffected dogs from eleven different countries. Genotyping using the Illumina high density canine single nucleotide variant genotyping chip was used to identify a candidate genetic region. There was a highly significant peak of association over chromosome 17, with a p-value of 2 × 10−13. Expression profiles and evolutionary conservation of candidate genes were assessed using public databases. Whole genome sequences of three dogs with glaucoma, three severely affected by goniodysgenesis and three unaffected dogs identified a missense variant in the olfactomedin like 3 (OLFML3) gene in all six affected animals. This was homozygous for the risk allele in all nine cases with glaucoma and 12 of 14 other severely affected animals. Of 67 reportedly unaffected animals, only one was homozygous for this variant (offspring of parents both with goniodysgenesis who were also homozygous for the variant). Analysis of pedigree information was consistent with an autosomal recessive mode of inheritance for severe goniodysgenesis (potentially leading to glaucoma) in this breed. The identification of a candidate genetic region and putative causative variant will aid breeders to reduce the frequency of goniodysgenesis and the risk of glaucoma in the Border Collie population.
The collection of Oriental art at the Vilnius Museum of Antiquities in 1862–1865
Authors: Būčys Žygintas 
Published: January 2019

Vilnius UniversityThe present article provides a discussion about the collection of Oriental art acquired from Kronstadt residents in 1862 and exhibited at the Vilnius Museum of Antiquities. This was the first public collection of its kind in Lithuania. For this reason, the article is devoted to an analysis of the meanings and provisions that the Lithuanian scholarly community sought to impart to the exhibits of this collection as well as how they were perceived by the society at the time. A detailed discussion about the content of the collection, its attribution and donors is provided.
The first Lahu (Muhsur) Christians: A community in Northern Thailand
Authors: Walker Anthony R. 
Published: January 2019

Universiti Brunei DarussalamBetween 10 to 20 per cent of all the Tibeto-Burman-speaking Lahu people now subscribe to one or another version of the Christian religion.The largest proportion of present-day Lahu Christians inherited the genre of this Western religion propagated by American Baptist missionaries in the former Kengtung State of Burma (from 1901 to 1966), in Yunnan (from 1920 to 1949), and in North Thailand (from 1968 to 1990). For this reason, it is often thought that pioneer American Baptist among the Lahu, William Marcus Young (1861–1936), was the first to induct a representative of this people into the Christian faith.In fact this is not the case. The first Lahu Christians lived in North Thailand, baptised by long-time Chiang Mai-based American Presbyterian missionary, Daniel McGilvary. This was in 1891, thirteen years before Young’s first baptism of a Lahu in Kengtung, Burma, in October 1904.The paper addresses three questions. Why were Lahu living in upland North Thailand in the early 1890s? Why did one small Lahu community decide to embrace the Christian religion? Finally, why, in stark contrast to Baptist Christianity in the Lahu Mountains, did this fledgling Lahu Presbyterian community disappear, apparently without trace, sometime after 1920?
Sentido de vida e saúde mental durante o tratamento de câncer
Authors: Santos Junior Randolfo , De Simone Caroline Santos , Pinhabel Gabriela Volpe , Galhardo Valeria Aparecida Teixeira , Carniel Aline 
Published: January 2019

Câncer é uma doença crônica de alta prevalência e que impõe desafios de ajustamento ao paciente e seus familiares. Os objetivos deste estudo foram avaliar a presença de sentido de vida em pacientes com câncer e sua correlação com outras variáveis psicossociais. Material e Método: Participaram 91 pacientes em tratamento oncológico há no mínimo três meses. Instrumentos: Questionário Sóciodemográfico; Escala Hospitalar de Ansiedade e Depressão; Escala de Esperança de Herth; Mini-Mac; Escala de Apoio Social. Resultados: Observa-se uma correlação positiva entre os indicadores de sentido de vida e os indicadores de esperança, apoio social, tempo de diagnóstico e rede de apoio. E uma correlação negativa com os indicadores de depressão, preocupação ansiogênica e desamparo. Conclusão: Há evidências de que a presença de sentido associada a outras variáveis é um possível indicador de proteção à saúde mental dos pacientes em tratamento de câncer.
O sublime e a coragem possível: uma contribuição do Grande Sertão: Veredas para a Literatura Brasileira
Authors: Oliveira Renato Rocha de , Cornelsen Élcio Loureiro 
Published: January 2019

Resumo: Este artigo aborda a influência de Diadorim sobre Riobaldo em GrandeSertão: Veredas, pela perspectiva filosófica do sublime e da coragem (Schiller e outros), baseando-se em concepções de Walter Benjamin e dialogando com a crítica de Willi Bolle. O ensaio identifica, na obra-prima de Guimarães Rosa, uma relação importante entre o sublime, associado a Diadorim a partir de elementos levantados por Benjamin, como a figura guia, o amor provençal e o justo hermafrodita, e a coragem demonstrada por Riobaldo. Finalmente, contribui para a avaliação de Bolle quanto ao caráter dialético e verossímil do texto literário no que concerne à coragem desse personagem, em comparação com a representação do sertanejo em Os Sertões, de Euclides da Cunha.Palavras-chave: Diadorim, figura guia, sublime, coragem, Benjamin, Bolle.Abstract: This article approaches the influence of Diadorim upon Riobaldo in The devil to pay in the backlands through the philosofical perspective of sublime and courage (Schiller and others), based on Walter Benjamin’s conceptions and in dialogue with Willi Bolle’s critique. The essay identifies, in Guimarães Rosa’s masterpiece, an important relation between the sublime, associated with Diadorim per elements raised by Benjamin, as the guide figure, the corteous love, the hermaphrodite just, and the bravery showed by Riobaldo. Finally, contributes to Bolle’s evaluation of the dialectical and verisimilar character of the literary text concerning this character’s courage, in comparison with the countryside representation in The Backlands, by Euclides da Cunha.Keywords: Diadorim, guide figure, sublime, courage, Benjamin, Bolle.
Cidade-montagem e cidade-confluência: As “Berlins” de Döblin e de Benjamin
Authors: Salles Anna Flávia Dias 
Published: January 2019

Resumo: Pretende-se, neste artigo, analisar aspectos dos distintos métodos narrativos com que os escritores Alfred Döblin e Walter Benjamin abordam a cidade de Berlim em suas obras, respectivamente, Berlin Alexanderplatz - a história de Franz Biberkopf, e Infância em Berlim por volta de 1900. Demonstra-se que ambos os autores logram rasurar a linha divisória entre espaços público e privado na cidade, reforçando a ideia de uma contiguidade indissociável entre estes nas obras em foco. Döblin pretende, com sua montagem literária de documentos, impregnar a Berlim ficcional de autenticidade, enquanto Benjamin se propõe, por meio de uma escrita fragmentária e errante, a abrir os acontecimentos singulares da vida citadina à dimensão histórica.Palavras-chave: representações da cidade, montagem literária, memória.Abstract: This article aims to analyze aspects of the distinct narrative methodswith which writers Alfred Döblin and Walter Benjamin approach the city of Berlinin their works, respectively, Berlin Alexanderplatz – the story of Franz Biberkopf,and Berlin Childhood around 1900. It is established that both authors manage to erase the dividing line between public and private spaces in the city, reassuring the idea of an inseparable contiguity between them in the focused works. Döblin intends, with his literary assemblage of documents, to impregnate the fictional Berlin with authenticity, while Benjamin proposes, through a fragmentary and erratic writing, to open the singular events of the city life to the historical dimension.Keywords: representations of the city, literary assemblage, memory.
Walter Benjamin e Roland Barthes às margens da escritura
Authors: Teixeira Derick Davidson Santos 
Published: January 2019

Resumo: A noção de escritura, desenvolvida por teóricos como Roland Barthes,Jacques Derrida e Maurice Blanchot, no século XX, possui um lugar medular nateoria da literatura e na crítica literária. O presente trabalho propõe um cotejamento entre a teoria de Walter Benjamin e de Roland Barthes no que concerne à escritura. Tomando alguns traços principais da escritura, analisados por Barthes, em conjunto com o pensamento de Benjamin acerca da narração, do declínio da experiencia e de algumas obras da literatura moderna – como a obra de Proust– é possível elucidar de que forma a escritura, operando como um limiar (Schwelle), escapa à rigidez das fronteiras que separam o pessoal e o histórico, a ordem comum do individualismo, a experiência da vivência.Palavras-chave: Roland Barthes; Walter Benjamin; escritura; limiar.Abstract: The notion of writing, developed by theorists such as Roland Barthes, Jacques Derrida and Maurice Blanchot, in the 20th century, has a fundamental place when it comes to literary theory and literary criticism. This work proposes a collating between Walter Benjamin’s and Roland Barthes’ theory concerning writing. Taking some main features of writing, analyzed by Barthes, together with Benjamin’s thought about narration, the decline of experience and some modern literary works – such as Proust’s oeuvre – it is possible to elucidate how writing, working as a threshold (Schwelle), escapes from the rigidity of the borders that separate the personal and the historical, the common order and individualism, experience and “inner lived experience”.Keywords: Roland Barthes; Walter Benjamin; writing; threshold.
Gündüz Kuşağı Kadın Programlarında Toplumsal Cinsiyet Eşitsizliği ve Kadın Temsili
Authors: ÇAVDAR Ceren 
Published: January 2019

Bu makalede Türk
toplumu içerisinde yer aldığı varsayılan sorunlu genel kabullerin kadına
yönelik gündüz kuşağı programlarında nasıl temsil edildiği incelenmiştir. Bu
bağlamda ülkemizde güncel izlenme oranları göz önüne alınarak 3 ulusal
televizyon kanalından 3 örneklem seçilmiştir. Atv’den “Müge Anlı’yla Tatlı Sert, Show Tv’den Seda
ve Uğur’la, Star Tv’den Balçiçek İlter’le Olay Yeri” isimli bu üç programın
rastlantısal olarak seçilerek her birinden 3 bölüm olmak üzere toplam 9 bölüme
nitel ve nicel içerik analizi yapılmıştır ve kadının toplumsal cinsiyet rolü
bağlamında temsili incelenmiştir. Araştırmada Mc Combs ve Shaw tarafında
geliştirilen gündem belirleme modeli kullanılmıştır. Bunun sebebi ise gündem
belirleme kuramında medyanın gündemi belirlediği genel kabulünden hareket
ettiğimizde, kadın programları da kadınların gündemini seçtiği konu ve
kategorilerle belirlemektedir. Araştırmanın sonucunda gündüz kuşağı kadın
programlarının ülkedeki var olan toplumsal cinsiyet eşitsizliğini pekiştirdiği
sonucuna ulaşılmıştır. Bu durumun pekiştirildiğini incelenen televizyon
programlarında özellikle Seda ve Uğur’la ve Müge Anlı’yla Tatlı Sert
örneklerinde görülmektedir. İncelenen örnekler doğrultusunda yayınlanan tüm
programların sorunlu olduğu söylenmemekle birlikte sorunlu söylemler olduğu
saptanmıştır. Fakat Balçiçek İlter’le Olay Yeri programı cinsiyetçi söylemlere
çok yer vermemekle birlikte cinsiyetçi yaklaşıma karşı olduğunu belirten
söylemlerde bulunmuştur.
Fransız Yeni Dalga Sineması İçinde Yenilikçi Bir Yönetmen Jean Luc Godard Filmlerinin İdeolojik Boyutu
Authors: YILDIRIM Erhan , CAN Aytekin 
Published: January 2019

Fransız sineması; dünya
sineması içinde sinemanın doğduğu günden bugüne kadar önemli bir yere sahiptir.
Fransız sinemasının dünya sinemasında önemli yere sahip olmasına ve bir ekol
olarak ortaya çıkmasına etki eden en önemli sebep, 1950’li yılların sonlarına
doğru Yeni Dalga sinema akımının ortaya çıkışıdır. Akım, sanat ile sanatçı arasındaki ilişkiye
farklı bir boyut getirmiştir. Eskiye elveda derken yeninin manifestosunu yazan
bir dalganın kuşağı sinema tarihine adını yazdırmıştır. Sadece Fransız
sinemasında değil tüm dünya sinemasında bir fenomene dönüşen Yeni Dalga;
sinemaya teorik, estetik ve teknik anlamda pek çok yenilik kazandırmıştır.
Godard, Truffaut, Rohmer, Resnais gibi pek çok yetenek ile parlak bir dönem
yaşayan Fransız sineması birçok ülkede sinema sektörünü etkileyen akıma
dönmüştür. Bu süreçte Fransız sinemasının politik yüzünün de ortaya çıktığı
görülür. Politik yüzünün de olduğu görülen bu oluşumun içinde farklı, isyankar,
yaratıcı, devrimci tarzı ve tavrı ile ortaya çıkan bir isim Jean Luc Godard
akımın önemli bir ismi olmuştur. Yapılan bu
çalışmanın amacı, Godard’ın Yeni Dalga Sinema Akımı ile başlayan yenilikçi
yüzünü Godard’ın ideolojik yapısı üzerinden izleyicisine sunabilmektir. Çalışmada
Godard’ın sinema kariyeri ve ideolojisinin çözümlemesi, Godard’ın iki önemli
filmi, Serseri Aşıklar ve Küçük Asker filmleri örnekleminde niteliksel
araştırmanın farklı kolları olan tarihsel, toplum bilimsel ve arketipçi
araştırma yöntemleri ile yapılmıştır.
1980-2017 Yılları Arasında Dijital Oyun Endüstrisinin Gelişimi ve Oluşturulan Yapay Yaşam Döngüleri
Authors: DİKMEN Ergin Şafak 
Published: January 2019

Bu çalışmanın amacı küresel dijital oyun endüstrisinin
gelişim sürecini anlamak ve dijital oyun
ekosisteminin bileşenleri arasındaki etkileşimi
çözümlemektir. Bu kapsamda dünyanın en büyük oyun firmaları arasından
yer alan Activision firmasının 1980 yılından itibaren piyasaya sürdüğü dijital
oyunlar incelenmiştir. Çalışmanın odaklandığı sorular şunlardır: Küresel oyun
endüstrisi nasıl bir gelişim patikası izlemiştir? Dijital oyunlar ve oyun
konsolları arasında ne tür bir ilişki bulunmaktadır? Geliştirilen oyunlar ile
kültür endüstrisindeki diğer sektörler arasında nasıl bir etkileşim
bulunmaktadır? Bu bileşenler nasıl bir yaşam döngüsüne sahiptir? Hangi dinamikler
ve stratejiler gözetilerek yeni oyunlar piyasaya sürülmektedir? Bu sorulara
yanıt bulmak amacıyla çalışma Activision firmasının geliştirdiği oyunların
yayımlanma tarihleri, geliştirilen oyun serileri, oyun konsollarının nesilleri ve
dijital oyunların ilişkili olduğu sinema ve televizyon öyküleri niceliksel
içerik analiz yöntemiyle çözümlenmiştir. Elde edilen sonuçlar doğrultusunda dijital
oyunların yapay yaşam döngülerinin oluşturulduğu ve bu oyunların düzenli
aralıklarla yeniden üretilerek piyasaya sürüldüğü belirlenmiştir. Bunun
sonucunda dijital oyunların; oyun yayımcıları, kültür endüstrisindeki farklı
sektörler ve konsol üreticilerinin dahil olduğu üç ayaklı karmaşık bir dijital
oyun ekosistemi içerisinde geliştirildiği ortaya konmuştur.
A NEEDS ANALYSIS OF CHINESE LANGUAGE TEACHING MATERIALS FOR STUDENTS OF BUSINESS AND MANAGEMENT MAJORS IN A VOCATIONAL HIGH SCHOOL
Authors: Fitria Ayu , Rasyid Yumna , Lustyantie Ninuk 
Published: January 2019

<p>The purpose of this study was to identify the Chinese language teaching materials for students of business and management majors in a vocational high school. This research method was qualitative. The process of collecting data used observation activities, interviews and questionnaires. This study found that the Chinese teaching materials which have been used in a vocational high school especially in business and management majors were still general and not relevant to the needs of students. The results of this study also indicated that the expected design of Chinese language teaching materials should be specifically integrated to the students’ majors and the competency levels of their expertise. In addition, the teaching materials also should be designed with multimedia to support the learning process and students’ experiences.</p><p><strong>BAHASA INDONESIA ABSTRAK: </strong>Penelitian ini bertujuan untuk mengidentifikasi kebutuhan model materi ajar bahasa Mandarin untuk Sekolah Menengah Kejuruan Bidang Bisnis dan Manajemen. Penelitian ini merupakan penelitian kualitatif dengan proses pengumpulan data melalui observasi, wawancara dan kuesioner. Melalui penelitian ini ditemukan bahwa materi ajar bahasa Mandarin yang selama ini digunakan di Sekolah Menengah Kejuruan Bidang Bisnis dan Manajemen merupakan materi ajar yang bersifat umum dan belum memenuhi kebutuhan siswa. Hasil dari analisis kebutuhan menunjukkan bahwa materi ajar bahasa Mandarin yang diharapkan adalah materi ajar yang terintegrasi dan relevan dengan bidang dan program studi siswa sehingga dapat mendukung keahlian utamanya. Terlebih lagi materi ajar bahasa Mandarin yang diharapkan saat ini adalah materi ajar yang dikemas dengan basis multimedia.</p>
Cross-Linguistic Influence in Chinese Learners of Two Foreign Languages While Studying Abroad in Spain
Authors: Zhao Wenxiao 
Published: January 2019

Studies in the area of cross-linguistic influence (CLI) have attracted the focus of multi-linguistic learners. However, little research on CLI deals with Asian learners, particularly Chinese-speaker with knowledge of two or more foreign languages. The present study explores CLI in L1 Chinese learners with both English (L2) and Spanish (L3) as foreign languages who are studying in Madrid, a Spanish-speaking community; their studies coincided with data collection. English learners were instructed to speak for analysis purposes, with the following aims: (i) to observe the most frequent category (functional transfer, code-switching, borrowing and coinage) in the CLI instances; (ii) to determine the source language of CLI; (iii) to investigate whether CLI factors, including language distance, L2 status, proficiency and recency of use, intervene in the appearance of CLI instances in the participants.
Data was gathered from 16 female Chinese students at Complutense University of Madrid (UCM). These were master students aged 22 to 26, who visited Spain for more than 5 months when they participated in the present study. The instrument used was an English semi-structured interview. Results primarily reveal that (a) borrowing is the most prevalent category, accounting for 70% of the CLI instances; (b) Spanish is the main source language of CLI while Chinese plays a functional role in the transfer process; (c) language distance proves to be the strongest predictor of CLI.
PARASITES IN GILLS OF Aequidens tetramerus, CICHLID FROM THE LOWER JARI RIVER, AN TRIBUTARY OF THE AMAZON RIVER, NORTHERN BRAZIL
Authors: Tavares-Dias Marcos , Felix Borges William , Gomes Santos Gracienhe , Sidney Brito de Oliveira Marcos 
Published: January 2019

The aim of this study was to investigate for the first time the parasite fauna in the Aequidens tetramerus gills of the lower Jari River, State of Amapá (Brazil). In January 2017, 31 specimens of A. tetramerus were collected and they were necropsied using usual methodologies for parasitology of fish. A total of 1,181 parasites were collect and 100% of hosts were parasitized by Gussevia disparoides, Gussevia alioides, Posthodiplostomum sp., Clinostomum sp., Dolops longicauda and Acarina gen. sp. The dominance was of G. disparoides and there was aggregated dispersion of G. disparoides, Posthodiplostomum sp. and Clinostomum sp., while the dispersion of G. alioides, D. longicauda and Acarina gen. sp was random. There were low species richness of parasites (2.45 Â± 0.68), low Brillouin diversity index (0.52 Â± 0.22), evenness (0.37 Â± 0.15) and high Berger-Parker dominance (0.70 Â± 0.15). The parasites community was characterized by low richness of species with high prevalence and low parasitic abundance. Host length had little influence on the abundance of parasites, but host behavior and availability of infective stages of parasites were the factors structuring the parasite community in A. tetramerus. This was the first study on parasites of A. tetramerus from the Jari River basin.