bluuebunny/crossref_metadata_2025_split huggingface dataset:
Inherited bone marrow failure associated with germline mutation of ACD, the gene encoding telomere protein TPP1
Authors: Guo Yiran , Kartawinata Melissa , Li Jiankang , Pickett Hilda A. , Teo Juliana , Kilo Tatjana , Barbaro Pasquale M. , Keating Brendan , Chen Yulan , Tian Lifeng , Al-Odaib Ahmad , Reddel Roger R. , Christodoulou John , Xu Xun , Hakonarson Hakon , Bryan Tracy M. 
Published: September 2014

AbstractTelomerase is a ribonucleoprotein enzyme that is necessary for overcoming telomere shortening in human germ and stem cells. Mutations in telomerase or other telomere-maintenance proteins can lead to diseases characterized by depletion of hematopoietic stem cells and bone marrow failure (BMF). Telomerase localization to telomeres requires an interaction with a region on the surface of the telomere-binding protein TPP1 known as the TEL patch. Here, we identify a family with aplastic anemia and other related hematopoietic disorders in which a 1-amino-acid deletion in the TEL patch of TPP1 (ΔK170) segregates with disease. All family members carrying this mutation, but not those with wild-type TPP1, have short telomeres. When introduced into 293T cells, TPP1 with the ΔK170 mutation is able to localize to telomeres but fails to recruit telomerase to telomeres, supporting a causal relationship between this TPP1 mutation and bone marrow disorders. ACD/TPP1 is thus a newly identified telomere-related gene in which mutations cause aplastic anemia and related BMF disorders.
Viewing systematic reviews and meta-analysis in social research through different lenses
Authors: Davis Jacqueline , Mengersen Kerrie , Bennett Sarah , Mazerolle Lorraine 
Published: September 2014

Abstract
Systematic reviews and meta-analyses are used to combine results across studies to determine an overall effect. Meta-analysis is especially useful for combining evidence to inform social policy, but meta-analyses of applied social science research may encounter practical issues arising from the nature of the research domain. The current paper identifies potential resolutions to four issues that may be encountered in systematic reviews and meta-analyses in social research. The four issues are: scoping and targeting research questions appropriate for meta-analysis; selecting eligibility criteria where primary studies vary in research design and choice of outcome measures; dealing with inconsistent reporting in primary studies; and identifying sources of heterogeneity with multiple confounded moderators. The paper presents an overview of each issue with a review of potential resolutions, identified from similar issues encountered in meta-analysis in medical and biological sciences. The discussion aims to share and improve methodology in systematic reviews and meta-analysis by promoting cross-disciplinary communication, that is, to encourage ‘viewing through different lenses’.
Response of airway epithelial cells to double-stranded RNA in an allergic environment
Authors: Herbert Cristan , Zeng Qing-Xiang , Shanmugasundaram Ramesh , Garthwaite Linda , Oliver Brian G , Kumar Rakesh K 
Published: September 2014

AbstractBackgroundRespiratory viral infections are the most common trigger of acute exacerbations in patients with allergic asthma. The anti-viral response of airway epithelial cells (AEC) may be impaired in asthmatics, while cytokines produced by AEC may drive the inflammatory response. We investigated whether AEC cultured in the presence of Th2 cytokines associated with an allergic environment exhibited altered responses to double-stranded RNA, a virus-like stimulus.MethodsWe undertook preliminary studies using the MLE-12 cell line derived from mouse distal respiratory epithelial cells, then confirmed and extended our findings using low-passage human AEC. Cells were cultured in the absence or presence of the Th2 cytokines IL-4 and IL-13 for 48 hours, then stimulated with poly I:C for 4 hours. Expression of relevant anti-viral response and cytokine genes was assessed by quantitative real-time PCR. Secretion of cytokine proteins was assessed by immunoassay.ResultsFollowing stimulation with poly I:C, MLE-12 cells pre-treated with Th2 cytokines exhibited significantly higher levels of expression of mRNA for the cytokine genesCxcl10andCxcl11, as well as a trend towards increased expression ofCxcl9andIl6.Expression of anti-viral response genes was mostly unchanged, althoughStat1, Ifit1andIfitm3were significantly increased in Th2 cytokine pre-treated cells. Human AEC pre-treated with IL-4 and IL-13, then stimulated with poly I:C, similarly exhibited significantly higher expression ofIL8, CXCL9, CXCL10, CXCL11andCCL5genes. In parallel, there was significantly increased secretion of CXCL8 and CCL5, as well as a trend towards increased secretion of CXCL10 and IL-6. Again, expression of anti-viral response genes was not decreased. Rather, there was significantly enhanced expression of mRNA for type III interferons, RNA helicases and other interferon-stimulated genes.ConclusionThe Th2 cytokine environment appears to promote increased production of pro-inflammatory chemokines by AEC in response to double-stranded RNA, which could help explain the exaggerated inflammatory response to respiratory viral infection in allergic asthmatics. However, any impairment of anti-viral host defences in asthmatics appears unlikely to be a consequence of Th2 cytokine-induced downregulation of the expression of viral response genes by AEC.
X-ray tomography of large wooden artworks: the case study of "Doppio corpo" by Pietro Piffetti
Authors: Re Alessandro , Albertin Fauzia , Avataneo Chiara , Brancaccio Rosa , Corsi Jacopo , Cotto Giorgio , De Blasi Stefania , Dughera Giovanni , Durisi Elisabetta , Ferrarese Walter , Giovagnoli Annamaria , Grassi Novella , Lo Giudice Alessandro , Mereu Paolo , Mila Giorgia , Nervo Marco , Pastrone Nadia , Prino Francesco , Ramello Luciano , Ravera Massimo , Ricci Chiara , Romero Alessandra , Sacchi Roberto , Staiano Amedeo , Visca Lorenzo , Zamprotta Lorenzo 
Published: September 2014

Abstract

Introduction
X-ray computed tomography (CT) is now used in the cultural heritage field because it is non-invasive and it can give a large amount of information on the inner structure of the object under study. Until recently mainly medical CT scanners or micro-CT setups have been used, limiting the analysis to relatively small artworks or requiring multiple acquisition and difficult image-joining for objects larger than detector dimensions.

Results
To overcome the limitations of ordinary CT devices, a facility for the X-ray tomography of large size artefacts has recently been designed and installed in a protected area of the Fondazione Centro Conservazione e Restauro "La Venaria Reale", a Centre for Preservation and Restoration. This facility, based on a X-ray source, a linear X-ray detector and a high precision mechanical system, has been and will be used to gather information on materials, manufacturing techniques and conservative conditions of artworks undergoing the restoration process.
In this paper the results of the tomography of the first analyzed large artistic object are presented, giving an idea of the wealth of information obtained from the CT scan. The presented artwork is the writing cabinet called "doppio corpo", a masterpiece of furniture more than 3 m high, inlaid for Savoy Residences by Pietro Piffetti, the most famous cabinet-maker in Piedmont in the XVIII century. The artwork is now housed in the Quirinale Palace, the official residence of the Italian President in Rome.

Conclusions
The CT analysis permitted us to obtain valuable information about the conservative conditions, the presence of previous interventions, the distribution of various materials and the dimensions and arrangement of several wooden pieces, thus allowing for interesting hypotheses about the building technique of this masterpiece.
A security analysis of uniformly-layered rainbow defined over non-commutative rings
Authors: Yasuda Takanori , Sakurai Kouichi 
Published: September 2014

Abstract
Hashimoto and Sakurai proposed a signature scheme (HS scheme), whose security is based on the difficulty of integer factorization. In this paper, we redefine HS scheme as a signature scheme in Multivariate Public Key Cryptosystems (MPKC). MPKC are public key cryptosystems whose security is based on the difficulty of solving multivariate quadratic equations, and candidates for post-quantum cryptography. In this paper, we analyze the security of the extended HS scheme using technique of security analysis for MPKC. Furthermore, based on the security analysis of the extended HS scheme, we estimate secure parameters of the extended HS scheme.
Using Twitter to investigate opinions about multiple sclerosis treatments: a descriptive, exploratory study
Authors: Ramagopalan Sreeram V. , Wasiak Radek , Cox Andrew P. 
Published: September 2014

Background: Multiple sclerosis (MS) is a common complex disorder, with new treatment options emerging each year. Social media is being increasingly used to investigate opinions about drugs, diseases and procedures. In this descriptive exploratory study, we sought to investigate opinions about currently available MS treatments.Methods: The Twitter resource Topsy was searched for tweets mentioning the following MS treatments: Aubagio, Avonex, Betaferon or Betaseron, Copaxone, Extavia, Gilenya, Lemtrada, Novantrone, Rebif, Tysabri and Tecfidera between 1 Jan 2006 to 31 Jul 2014. Tweets were normalised and sentiment analysis performed.Results: In total, there were 60037 unique tweets mentioning an MS treatment. About half of the tweets contained non-neutral sentiment. Mean sentiment scores were different for treatments ranging from -0.191to 0.282 when investigating all tweets. These differences in sentiment scores between treatments were statistically significant (P<0.001). Sentiment scores tended to be higher for oral MS treatments than injectable treatments.Conclusions: Many tweets about MS treatments have a non-neutral sentiment. The analysis of social media appears to be a potential avenue for exploring patient opinion about MS treatments.
W okowach „demokratycznego centralizmu” – przypadek Stronnictwa Demokratycznego w PRL
Authors: Tyszkiewicz Adrian 
Published: September 2014

In the grip of “democratic centralism” – the case of The Democratic Party in The People Republic of Poland
So – called “democratic centralism”, originally created by W. Lenin, was the main principle used by the communist party (PPR/PZPR) in Poland after the World War II to control the society and its organizations. One of them was a Democratic Party established in the 1939 by the politicians and activists rooted in the left – oriented independence movement, of whom Marshall Józef Piłsudski said to has been the icon and the undisputed leader. After the war the Democratic Party was enslaved by the communists and became the part of the rule system. It was said to be the representative of the systematically vanishing middle class (craftsmen, merchants, intelligence), due to the thesis of Stalin concerning the raging class struggle. Until the fall of the totalitarian system in 1989 the Democratic Party officially supported the communists who overwhelmed every aspect of political reality. That is why the phenomenon of political realism was not involved in its policy despite of being the part of the official propaganda. Nevertheless, in the party structures dwelled the resistance that headed for the independence, of whom Karol Głogowski or Przemysław Górny were the leaders. The Democratic Party conversed in 1989 and supported the first democratic government of Tadeusz Mazowiecki.
Realizm polityczny „Tygodnika Powszechnego” (1945-1989)
Authors: Graczyk Roman 
Published: September 2014

The political realism of ,,Tygodnik Powszechny” (1945-1989)
In the history of „Tygodnik Powszechny” under the administration of the communist government, I distinguish four periods – each one characterized by a different attitude of its environment to communism. The first one (1945‑1948) was dominated by the aim to create an ideological alternative towards the new power; the second one (1949‑1953) in which the goal of survival, in the face of communism’s inevitable victory, prevailed; the third one (1956/7-1976), characterized by willingness to cohabitate and limited collaboration; and the fourth one (1977‑1989), which saw the exit from People’s Republic of Poland communist system. Glancing on this history through the prism of political realism, one can establish, that „Tygodnik” disregarded its guidelines in the first period (1945‑1948), tried to fulfill them – in a very original way nonetheless – in the second period (1949‑1953), and finally became its eulogist, having created its own doctrine of catholic political realism in the actuality of a communist state, in the third one (1956/7‑1976). The fourth period (1977‑1989), however, is difficult to classify in this aspect.
Realizm z fałszywej analogii historycznej wywiedziony
Authors: Kimla Piotr 
Published: September 2014

Realism based on the false historical analogy. A case of Ksawery Pruszyński
The article focuses on Ksawery Pruszyński’s political way of thinking which squares with political realism in many points. He aimed at developing Poles’ awareness of the fact that Stalin would be the winner of the war in Central and Eastern Europe. Therefore the future status of Poland depended on Stalin’s will. Nethertheless Pruszyński seems to make a serious mistake taking Soviet Russia as an atheistic, Marxist version of Tsarist Russia. Yet as things stand the Soviet Union was a phenomenon of a new kind. Ideocracy is presumably the best qualification of this new regime.
Realizm polityczny i ekonomiczny Mirosława Dzielskiego
Authors: Kuniński Miłowit 
Published: September 2014

Political and economic realism of Mirosław Dzielski
Mirosław Dzielski (1941‑1989), a Polish philosopher and a political activist, in his academic life at the Jagiellonian University (Krakow) he focused on analyses of metaphysical subtleties of Newton’s and Leibniz’s conceptions of space and time but his main interest were the major problems of ethics and political philosophy combined with economics. Influenced by L. von Mises, F. A. von Hayek and M. Friedman and the idea of the undesigned results of human activities, he identified the main problem of his native country Poland and the whole region under a totalitarian control of the USSR in the 1970s and 1980s, as a search for forces, which would stop its civilizational collapse and begin its rebirth. His idea was to destroy the oppressive system in an evolutionary way through the invigoration of natural propensities to improve one’s situation. Members of the Communist nomenklatura were no exception, and their involvement in economic activities backed by the free market legal framework were to open opportunities to all members of society (individual liberty) and to lead to the economic and civilizational progress followed by a political liberal constitution.
Prawnokarne ograniczenie wolności wypowiedzi w Polsce (wybrane aspekty)
Authors: Czarny‑Drożdżejko Elżbieta 
Published: September 2014

Penal limitation on freedom of expression in Poland (selected aspects)
The article titled “The Criminal Law restrictions on freedom of expression in Poland” concerns the issue of selected criminal offences to the press. Many legal rights must be protected by the norms and mores. Thus, journalists, and editors‑in‑chief must be aware of the possible criminal liability for violations. These regulations limit the freedom of expression, which, however, is not absolute. I think that the penalization of the abuse of freedom of expression is essential in today’s society. However, it is the anachronism to predict, in some cases (e.g. defamation, insults), in relation to the press, the possibility of applying the imprisonment penalty. Therefore, I believe, that it is necessary to liberalize the Polish criminal law.
Konserwatyzm bez metafizyki
Authors: Rysiewicz Mikołaj 
Published: September 2014

Conservatism without metaphysics. The case of Konstanty Grzybowski’s interwar thought
The aim of this article is the reconstruction and analysis of the political ideas that may be conceived as the components of Konstanty Grzybowski’s (Polish conservative thinker of the interwar period) conservative attitude. Grzybowski’s case is one of the most interesting among other conservatives in this period due to at least three reasons: his reluctant attitude towards Catholic Church, his acceptation for democracy (very rare among most of the Polish conservatives) and also his controversial decisions on his political affiliations in the interwar (but also postwar) period in Poland. The authors claim is that Grzybowski’s “peculiarity” results from the ideas that shaped his conservative attitude: the acceptation of political evolution, political realism and republicanism. This combination may be understood only by taking into closer consideration his philosophical background, especially the resignation of any metaphysical foundations of the political power that may follow from religious beliefs.
Wybrane problemy międzynarodowej integracji gospodarczej na obszarze postradzieckim w latach 1991-2010
Authors: Gafarowski Dymitr 
Published: September 2014

Selected issues of international economic integration in the post‑Soviet space in the period of 1991‑2010. A Russian perspective
The article focuses on the evolution, dynamics and structure of international economic integration in the post‑ Soviet space in the period of 1991-2010. The main objective of the article is to review synthetically the most crucial issues of integration processes from political and economic perspectives in the region under question. In the reality of globalization, the post‑ Soviet countries are forced to make attempts in order to get into the stream of its processes by means of creating economic integration unions which are able to react effectively to the changes. However, the political implication behind economic integration processes as well as the search for the identity of new independent states made them very different from each other, which is reflected in the employment of various models of national economies, political systems and the pace of development. Therefore the leading integration projects appeared to be generally ineffective. A look into the rich Russian literature concerning the topic allowed to reflect the Russian perspective on the processes which are taking place.
Ruch turystyczny w Peru w latach 1989-2006
Authors: Gruszczyński Mikołaj 
Published: September 2014

Tourism in Peru between 1989-2006
Tourism in Peru has been thriving for over 20 years. Tourism as the mechanism of the interdependence along with numerous cooperating institutions, new archaeological discoveries and researches uses prosperity of regions being based among others on a legacy of Incas. I will concentrate on statistical data concerning firstly on the arrival of tourists to Peru in 90ies and secondly on the arrival of tourists to Peru and neighbouring countries of Ecuador and Bolivia, thirdly the increase of tourist accommodation towards the development of tourists movements in years 1993-2003 . I will specify the objects in Peru marked out on the UNESCO list. I will wonder what role in tourism the autochthonous resident is playing, his relations with the cultural legacy of Incas and possibilities of participating in the life of tourism. Tourist movements before 2000 and precipitated development of tourism after 2000, its causes and the infrastructure which rose along with it.
Świadomość grzechu i poczucie świętości jako dwa bieguny tożsamości prawosławnych Rosjan
Authors: Kudroń Jakub 
Published: September 2014

Consciousness of sinfulness and a sense of holiness as two opposite aspects of Russian orthodox Christian identity
We are convinced that it is impossible to indicate and to describe one leading and homogeneous type of identity, which would be common for majority of Russian Federation citizens in the beginning of XXI century. Since the reign of tzsar Peter I, Russian nation and society have been strongly divided into fractions of the followers of western European culture influences, and their conservative opponents, radically devoted to the local traditions. After the Bolshevik revolution and the soviet period situation turned even more complicated, as the attitude towards communism became another factor of national split. In this article we decided to concentrate on the specific type of old‑ Russian, orthodox identity, often named as holly Russia identity – most typical for early Russian state and strongly suppressed by so‑ called modern political movements as well as by communists. We are indicating three foundations of holly Russia identity, which are: language, national and religious community and the Christian orthodox state. All these factors are deeply rooted into religious ground, making holly Russia people orthodox believers, creating relatively harmonious civilization of spiritual, material, legal and political culture built on the same religious basis. This order has been violently destroyed by the Bolshevik revolution and the soviet state. When the Soviet Union collapsed holly Russia tradition was legalized back. Since the election of Vladimir Putin it has been also included into official Russian political order. Unfortunately it is very likely, that Vladimir Putin, former KGB officer is only using traditional symbols as the PR and political weapon.
Tożsamość miejsca
Authors: Banaszkiewicz Magdalena 
Published: September 2014

The identity of the place. The case of Sankt Petersburg
The paper is focusing on the relationship between space and identity. According to the term genius loci, which may be understood as location’s distinctive atmosphere or a “spirit of place”, it is possible to explore some visible and invisible features of the place which play substantial role in social life of the local community. The case study of Saint Peterburg illustrates how the context of place, where people live, can have a significant influence on how they define themselves. The article raises the subject of cultural interpretation of space and compares the past and the present identity of St. Peterburg inhabitants. It focuses on myths, images and crucial events from the history, which constitute memory, value-system and social practices of modern citizens.
Radziecka wspólnota dziecięca – odsłona powojenna
Authors: Anna Kadykało  
Published: September 2014

Советское детское сообщество – послевоенная картина (на основании воспоминаний Алексея Козлова Козел на саксе)
Аналитическим материалом в данной статье являются воспоминания популярного советского и русского саксофониста Алексея Козлова Козел на саксе. На их основе можно рассматривать процесс формирования тождественности, сочетающий различные средовые влияния. В статье указаны факторы, влияющие на сознание поколения, детство которого пришлось на военные и послевоенные годы. Пространством, в котором дети проводили свою внешкольную жизнь, был двор, составляющий пример коллективного участия в культуре повседневности. Дворовая жизнь отличалась множеством игр – спортивных, азартных, дифференцированных по степени опасности. Особенное место среди факторов, влияющих на сознание дорастающего поколения, занимала широко распространенная культура преступного мира. Типичное для послевоенного детства общественное участие во внешкольной жизни прекращалось в период адолесценции, когда все большее значение отводилось индивидуализму и собственным увлечениям, которые считались путем для проявления исключительности личности. Стремление к индивидуализму, составляющее неотъемлемую черту периода взросления, в советских условиях усиливалось благодаря появлению альтернативной культуры (стиляги), являющейся интересным отличием от серой, обыденной советской действительности.