92 research outputs found
NPHP1 gene deletion is a rare cause of Joubert sindrome related disorders
No full textJoubert syndrome (JS) is a neurological disorder
characterised by a complex cerebellar and brainstem
malformation, the so called ‘‘molar tooth sign’’ (MTS).
JS can be associated with several abnormalities in
other organs, identifying a large spectrum of ‘‘Joubert
syndrome related disorders’’ (JSRD). Isolated nephronophthisis
(NPH) is an autosomal recessive tubulointerstitial
medullary cystic kidney disease, which can be
found in some JSRD. Among the four genes responsible
for isolated NPH (NPHP1–4), NPHP1 deletions have
been found in two families with JS plus NPH.
N We tested 40 JSRD probands with proven MTS for
NPHP1 deletions. Homozygous deletions were tested
by performing two multiplex PCR with two microsatellite
markers (one control marker and one internal
deletion marker) resolved on agarose gel. Five markers
within the common NPHP1 deletion region were
genotyped to test heterozygous deletions.
N A single NPHP1 homozygous deletion was found in a
patient presenting with cerebellar, retinal, and kidney
involvement, while heterozygous deletions were
excluded in the others. The appearance of MTS in the
patient with NPHP1 deletion was characteristic with
moderate cerebellar vermis hypoplasia and elongated
but not thickened superior cerebellar peduncles.
N We confirm that NPHP1 deletions can be a rare cause
of JSRD and broaden the NPHP1 associated clinical
spectrum. In all NPHP1 JSRD patients so far reported,
the MTS shows remarkably similar features, which
might be specifically associated with NPHP1 deletions
Measuring and Analyzing the Liquidity of the Italian Treasury Security Wholesale Secondary Market
Get PDFAlthough its importance, only recently the issue of liquidity in Treasury markets has received greater attention. We survey the literature about market liquidity and liquidity measures, and we put forward new measures. The aim is to provide a description of the liquidity of the Italian wholesale secondary market, which we describe thoroughly. We apply a large set of measures on a unique dataset, which gives us a complete view of the market. Even though the market provides an amount of liquidity that fits the market needs, the quality of the order book is low, and despite the presence of a large number of market makers, the degree of competition among them is not very high. Moreover, no clear and general relationship emerges between trading and order book measures. Indeed, even though trading activity is higher for on-the-run securities with respect to the off-the-run securities, there is not a sharp difference in terms of liquidity of the order book between them. In this case market regulation plays an important role. Finally, we investigate how long it takes for a new issue to become the benchmark for its segment. Our evidence shows that some modifications of the issuance policy in order to have a larger outstanding since the first auction could help securities in gaining earlier their benchmark status, especially in case of 10-year BTPs.Liquidity, liquidity measures, Government securities, market microstructure, benchmark status.
Does Market Transparency Matter? a Case Study
Get PDFWe analyse a change in the degree of transparency of MTS, the electronic inter-dealer market for Italian Government bonds, namely the July 1997 move to the anonymity of quotes. Our evidence supports the hypothesis that a decrease in transparency makes liquidity traders worse-off, whereas large/informed traders find it less costly to execute block trades. The evidence is also consistent with the “waiting game” hypothesis of Foster and Viswanathan (1996): under anonymity, traders tend to delay their trades in an attempt to acquire information through the order flow. From a public welfare perspective, our results indicate that the move to anonymity has been accompanied by an increase in market liquidity and by a reduction in volatility, a phenomenon that is also partly explained by the growth in Italy’s prospects for early participation in the EMU. The speed of information aggregation on MTS increases, as shown by an improvement of the MTS lead over the futures market. In a European perspective, the current organisation and performance of MTS place the market in a competitive position with respect to other sovereign bond markets and may contribute to their integration under the single currency.Electronic trading, market efficiency
Adomo's physiognomical image of Mahler: the convergence of music, painting, and language
Get PDFThis study makes a case for the manifestation of mannerism in the music of Mahler through a close reading of Adomo's monograph on the composer, concurrently supporting the theory that mannerism is a distinct style, not limited to fixed periods, conditions in art, or media. The label 'Mannerism' connotes the style of sixteenth century Italian fine art, circumscribed by various art historians in the twentieth century. This study argues that the style is evident beyond the constraints of the sixteenth century, through the investigation of its manifestations in different artworks, created both in earlier and more contemporary times. The argument is constructed from a detailed comparison of the characteristics of the style in the paintings of sixteenth century Italian artists Arcimboldo and Parmigianino, the early twentieth century music by Schoenberg and Mahler, and Virginia Woolfs last novel. The comparison is facilitated by the utilisation of Barthes's writings on Arcimboldo, John Ashbery's poem about one of Parmigianino's paintings, and, predominantly, Adomo's interpretation of Mahler. The study also addresses issues that concern a comparison between different media, such as the problematical nature of the convergence of the arts. For example, the comparison of linguistic elements in both Arcimboldo's and Mahler's artworks is difficult to conduct without implying that art or music become language; the notion of a painterly language, or a musical language is complex and ambiguous. The study deals with the issue of whether one medium has to be fundamentally similar to another, in order to identify common characteristics between the two. In accordance with Adorno's writings on this paradigm, the conclusion drawn supports the position that the style of mannerism can be identified as manifesting itself in different mediums, without the necessity to scrutinise the fundamental connection between music, painting and literary forms
Analisis Silabus Kurikulum 2013 Mata Pelajaran Fikih MTs Kelas VII
Get PDFThis study uses a qualitative research approach with document analysis methods. In the process, the authors sort and group data into themes. The results of the grouping are then interpreted and evaluated. The object of the author's study is the syllabus of Fiqh for Class VII of MTs. Curriculum 2013 revised edition 2016. Aspects targeted by the author include the structure, KD pairs, number of hours, learning activities, and assessment strategies. The results of the analysis provide conclusions; (1) There is an unsynchronization between syllabus, teacher's book and student's book. (2) There is an important component that is not in the syllabus, namely the learning objectives. (2) The distribution of time allocation is still uniform in one column even though the level of concept and practice in each material weighs differently, one material weighs heavier concept while other material has heavier practice weight, and d). The main material in the syllabus is incomplete, still too global so it is difficult to translate
EKSPLORASI KEMAMPUAN BERPIKIR KRITIS SISWA DALAM PEMBELAJARAN IPS DI MTS SKB 3 MENTERI
No full textThe lack of critical thinking of students, especially social studies subjects at MTs SKB 3 Minister Lubuk Bayas Perbaungan District is certainly caused by various factors, one of which is the lack of learning models applied by teachers to improve students' critical thinking skills. Based on the explanation above, the author feels the need to examine the problem and make it as a final project with the title “Critical Thinking Ability of Students in Social Studies Learning at MTs SKB 3 Minister Lubuk Bayas Perbaungan District. The purpose of this study was to determine whether or not the critical thinking skills of students in social studies learning at MTs SKB 3 Minister Lubuk Bayas Perbaungan District. Efforts made in improving students' critical thinking skills in social studies learning at MTS SKB 3 Minister Lubuk Bayas Perbaungan Subdistrict is to use effective methods to adapt the method to the material being taught and the method of question and answer and group discussion. Thus the critical thinking skills of students will increase
Formation of ring-shaped microtubule assemblies through active self-organization on dynein
Get PDFMicrotubule (MT)-kinesin, a biomolecular motor system, is a promising candidate for construction of artificial biomachines for a variety of nanotechnology applications. An active self-organization (AcSO) method involving a specific streptavidin (St)-biotin (Bt) interaction has been developed to assemble MTs into a highly ordered structure by exploiting their motility on a kinesincoated surface. Dynein is another biomolecular motor that moves along the MTs in the opposite direction from kinesin. Dynein has not yet been used to demonstrate the AcSO of MTs. In this study, we report the first successful demonstration of the AcSO of MTs on a dynein-coated surface to produce ring-shaped MT assemblies similar to those of kinesin. We found that ring-shaped MT assemblies obtained on dynein showed equal clockwise and counterclockwise rotational motion. This work will enrich the building blocks for designing future oriented motor protein-based artificial devices
A comparative study of machine translation engines: transliterating Russian anthroponyms and toponyms into Italian
Get PDFopenNowadays, enabling the transmission of knowledge and information among people belonging to different cultures is fundamental. Therefore, there is an always rising request of translations. To address this rising demand, translators in the past decades have started to rely more on tools that can aid their job: machine translation (MT) and computer-assisted translation tools (CAT), which are now essential. However, when it comes to languages that are quite distant from each other, such as Russian and Italian, finding words in the SL that do not have an attested translation in the TL can create some difficulties. In this case, one of the strategies that can be adopted is transliteration. Even MT has to perform transliteration when processing those words, such as proper names and toponyms. One of the main problems is that the outputs across various MTs do not always correspond, producing TWs with different characters that do not allow for a standardized transliteration. In this thesis an analysis on the accuracy of MT outputs is provided, so to assess which perform better among Language Waver, ModernMT, Intento, DeepL and Yandex when transliterating Russian anthroponyms and toponyms into Italian, taken from Russian passports. The metric of the assessment is based on a comparison between the output of each word generated from MTs and the norms suggested by the Russian Ministry of Foreign Affairs for anthroponyms and the UNGEGN Working Group for toponyms. Moreover, further investigation is devoted to ChatGPT-4Omni and its processing of selected STs to assess the translation outputs produced after receiving specific instructions in the prompt
Tectonic significance of changes in post-subduction Pliocene-Quaternary magmatism in the south east part of the Carpathian-Pannonian Region
Get PDFThe south-eastern part of the Carpathian–Pannonian region records the cessation of convergence between the European platform/Moesia and the Tisza–Dacia microplate. Plio-Quaternary magmatic activity in this area, in close proximity to the ‘Vrancea zone’, shows a shift from normal calc-alkaline to much more diverse compositions (adakite-like calc-alkaline, K-alkalic, mafic Na-alkalic and ultrapotassic), suggesting a significant change in geodynamic processes at approximately 3 Ma. We review the tectonic setting, timing, petrology and geochemistry of the post-collisional volcanism to constrain the role of orogenic building processes such as subduction or collision on melt production and migration. The calc-alkaline volcanism (5.3–3.9 Ma) marks the end of normal subduction-related magmatism along the post-collisional Călimani–Gurghiu–Harghita volcanic chain in front of the European convergent plate margin. At ca. 3 Ma in South Harghita magma compositions changed to adakite-like calc-alkaline and continued until recent times (< 0.03 Ma) interrupted at 1.6–1.2 Ma by generation of Na and K-alkalic magmas, signifying changes in the source and melting mechanism. We attribute the changes in magma composition in front of the Moesian platform to two main geodynamic events: (1) slab-pull and steepening with opening of a tear window (adakite-like calc-alkaline magmas) and (2) renewed contraction associated with deep mantle processes such as slab steepening during post-collisional times (Na and K-alkalic magmas). Contemporaneous post-collisional volcanism at the eastern edge of the Pannonian Basin at 2.6–1.3 Ma was dominated by Na-alkalic and ultrapotassic magmas, suggesting a close relationship with thermal asthenospheric doming and strain partitioning related to the Adriatic indentation. Similar timing, magma chamber processes and volume for K-alkalic (shoshonitic) magmas in the South Apuseni Mountains (1.6 Ma) and South Harghita area at a distance of ca. 200 km imply a regional connection with the inversion tectonics
The Development of Submerged Floating Tunnels as an innovative solution for waterway crossings
No full textThe present Thesis is organized in 10 chapters. In particular Chapter 1
gives a general overview of the modern solutions and technologies available
in the field of waterway crossings, introducing briefly the revolutionary
concept of Submerged Floating Tunnel.
Chapter 2 provides a deeper insight into the main characteristics of this
innovative structural solution for waterway crossings: first its structural
features are described and the relevant loading conditions discussed,
subsequently the main advantages of the SFT with respect to the traditional
crossing solutions, such as the Cable Supported Bridges and the Underground
and Immersed Tunnel are described.
Chapter 3 traces the history of the SFT, starting from its first proposal
made in 1969 for the Messina Strait crossing, describing all the feasibility
studies and preliminary designs developed all over the world in the following
years. This Chapter is concluded by a description of the Sino-Italian
cooperation programmes, involving among the other partners the University
of Naples “Federico II”, which led to a feasibility study relative to the
crossing of the Jintang Strait (P.R. of China) with a SFT and to the complete
design of the first SFT full-scale prototype, planned to be realized in the
forthcoming years in the Qiandao Lake (P.R. of China). A potential SFT
crossing in the Pulau Seribu Archipelago (Indonesia), preliminary studied in
the last years by the research team headed by Prof. Mazzolani, is also briefly
illustrated.
Chapter 4 explains in the detail of the various aspects faced in the design of
the Archimedes Bridge prototype. The features of the selected location and the
structural scheme are illustrated. The structural analyses aimed at
investigating the prototype behaviour under the environmental loads are
discussed. The conception and the design of the constructional details are
presented. Finally, the fabrication and erection procedures are briefly
described.
Chapter 5 provides a description of the structural models which can be used
to analyse the SFT structural behaviour: the beam on elastic foundation, which
2 Outline of the Thesis
can be used in the preliminary phase of the design, and a SFT Finite Element
Model.
Chapters 6 and 7 are devoted to the study of the response of the SFT to the
main environmental loads to which is subjected: the hydrodynamic actions
due to the presence of waves and currents and the earthquakes. Numerical
analyses are carried out with the Finite Element analyses aimed at the
understanding of the SFT structural behaviour and at the definition of the
optimal structural configurations.
In Chapter 8 potential SFT solutions developed for the Messina Strait and
Gibraltar Strait crossings are illustrated and a technical-economical
comparison with the Suspension Bridges designed for the same locations is
made.
Chapter 8 describes a simple procedure for a quick comparison of the SFT
and CSIB solutions with the Cable Supported Bridges one, providing useful
curves highlighting the conditions under which the former ones are more
competitive than the latter ones.
Finally, in Chapter 9 the future steps and challenges to be faced in the
development of Submerged Floating Tunnel are presented. An alternative
typology of floating tunnel is introduced too: the Cable Supported Immersed
Inversed Bridge (CSIB), which is conceived as a combination of the
submerged floating bridge concept with the cable system configurations and
features several advantages also with respect to the “traditional” SFT solution
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