37 research outputs found

    Coils and power supplies design for the SMART tokamak

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    Agredano-Torres, M., et al.A new spherical tokamak, the SMall Aspect Ratio Tokamak (SMART), is currently being designed at the University of Seville. The goal of the machine is to achieve a toroidal field of 1 T, a plasma current of 500 kA and a pulse length of 500 ms for a plasma with a major radius of 0.4 m and minor radius of 0.25 m. This contribution presents the design of the coils and power supplies of the machine. The design foresees a central solenoid, 12 toroidal field coils and 8 poloidal field coils. Taking the current waveforms for these set of coils as starting point, each of them has been designed to withstand the Joule heating during the tokamak operation time. An analytical thermal model is employed to obtain the cross sections of each coil and, finally, their dimensions and parameters. The design of flexible and modular power supplies, based on IGBTs and supercapacitors, is presented. The topologies and control strategy of the power supplies are explained, together with a model in MATLAB Simulink to simulate the power supplies performance, proving their feasibility before the construction of the system.This work received funding from the Fondo Europeo de Desarollo Regional (FEDER) by the European Commission under grant agreement numbers IE17-5670 and US-15570. Furthermore, it has been carried out within the framework of the EUROfusion Consortium and has received funding from the Euratom research and training programme 2014–2018 and 2019–2020 under grant agreement no. 633053. The views and opinions expressed herein do not necessarily reflect those of the European Commission

    Tumours in the pineal region in the paediatric age: Reports of 23 cases and a review of the literature

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    Introducción. Los tumores de la región pineal se localizan en una encrucijada de estructuras neurovasculares de difícil acceso quirúrgico y limitadas posibilidades de resección; por ello, el manejo de estas lesiones habitualmente requiere la asociación de tratamiento adyuvante radioterápico y/o quimioterápico. Pacientes y métodos. Análisis retrospectivo de las características epidemiológicas, clínicas, neurorradiológicas y anatomopatológicas de 23 pacientes con tumores de la región pineal tratados entre los años 1997 y 2010 en el Hospital Infantil Niño Jesús. Se discuten los factores implicados en el pronóstico de esta cohorte tras el tratamiento quirúrgico o adyuvante. Resultados. El estudio incluyó 6 niñas y 17 niños con edades comprendidas entre los 4 meses y 18 años. El 95% de los pacientes comenzó con signos de hidrocefalia aguda o subaguda, que precisaron la implantación de una derivación ventriculoperitoneal (82%). Se obtuvo una muestra histológica tumoral en todos los casos. Cinco pacientes fueron biopsiados y 18 fueron intervenidos mediante craneotomía. El germinoma (ocho casos) y el teratoma maduro (un caso) fueron los tumores con mayor supervivencia; los tumores no germinomatosos (tres casos), del parénquima pineal (cuatro casos) y gliomas (cinco casos) presentaron mayor tasa de recidiva y un peor pronóstico. Conclusión. El estudio de marcadores tumorales permite orientar el diagnóstico de determinados tumores de la región pineal. Actualmente, se recomienda realizar una toma histológica tumoral para establecer un diagnóstico preciso y un tratamiento oncológico específicoIntroduction. Tumours in the pineal region are located at a meeting point of several neurovascular structures that are difficult to reach surgically and for which the possibility of resection is limited; as a result the management of these lesions usually requires associated adjunctive treatment with radiotherapy and/or chemotherapy. Patients and methods. This study is a retrospective analysis of the epidemiological, clinical, neuroimaging and pathological characteristics of 23 patients with tumours in the pineal region who were treated between the years 1997 and 2010 in the Hospital Infantil Niño Jesús. The factors involved in the prognosis of this cohort following surgical or adjunctive treatment are also discussed. Results. Subjects included in the study were 6 girls and 17 boys with ages ranging from 4 months to 18 years. It was found that the initial symptoms in 95% of the patients were signs of acute or subacute hydrocephalus, which required the placement of a ventriculoperitoneal shunt (82%). A histological sample of the tumour tissue was collected in all cases. Biopsy samples were taken in the case of five patients and 18 underwent surgery involving a craniotomy. Germinoma (eight cases) and mature teratoma (one case) were the tumours with the longest survival times; non-germinomatous tumours (three cases), those of the pineal parenchyma (four cases) and gliomas (five cases) presented the highest rates of recurrence and a poorer prognosis. Conclusions. The study of tumour markers can be used to guide the diagnosis of certain tumours of the pineal region. At present, the recommended procedure involves taking a histological sample of the tumour in order to establish an accurate diagnosis and a specific oncological treatmen

    Importance of Post-Translational Modifications for Functionality of a Chloroplast-Localized Carbonic Anhydrase (CAH1) in Arabidopsis thaliana

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    Background: The Arabidopsis CAH1 alpha-type carbonic anhydrase is one of the few plant proteins known to be targeted to the chloroplast through the secretory pathway. CAH1 is post-translationally modified at several residues by the attachment of N-glycans, resulting in a mature protein harbouring complex-type glycans. The reason of why trafficking through this non-canonical pathway is beneficial for certain chloroplast resident proteins is not yet known. Therefore, to elucidate the significance of glycosylation in trafficking and the effect of glycosylation on the stability and function of the protein, epitope-labelled wild type and mutated versions of CAH1 were expressed in plant cells. Methodology/Principal Findings: Transient expression of mutant CAH1 with disrupted glycosylation sites showed that the protein harbours four, or in certain cases five, N-glycans. While the wild type protein trafficked through the secretory pathway to the chloroplast, the non-glycosylated protein formed aggregates and associated with the ER chaperone BiP, indicating that glycosylation of CAH1 facilitates folding and ER-export. Using cysteine mutants we also assessed the role of disulphide bridge formation in the folding and stability of CAH1. We found that a disulphide bridge between cysteines at positions 27 and 191 in the mature protein was required for correct folding of the protein. Using a mass spectrometric approach we were able to measure the enzymatic activity of CAH1 protein. Under circumstances where protein N-glycosylation is blocked in vivo, the activity of CAH1 is completely inhibited. Conclusions/Significance: We show for the first time the importance of post-translational modifications such as N-glycosylation and intramolecular disulphide bridge formation in folding and trafficking of a protein from the secretory pathway to the chloroplast in higher plants. Requirements for these post-translational modifications for a fully functional native protein explain the need for an alternative route to the chloroplast.This work was supported by the Swedish Research Council (VR), the Kempe Foundations and Carl Tryggers Foundation to GS, and grant numbers BIO2006-08946 and BIO2009-11340 from the Spanish Ministerio de Ciencia e Innovación (MICINN) to A

    Assessment of plasma chitotriosidase activity, CCL18/PARC concentration and NP-C suspicion index in the diagnosis of Niemann-Pick disease type C: A prospective observational study

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    Background: Niemann-Pick disease type C (NP-C) is a rare, autosomal recessive neurodegenerative disease caused by mutations in either the NPC1 or NPC2 genes. The diagnosis of NP-C remains challenging due to the non-specific, heterogeneous nature of signs/symptoms. This study assessed the utility of plasma chitotriosidase (ChT) and Chemokine (C-C motif) ligand 18 (CCL18)/pulmonary and activation-regulated chemokine (PARC) in conjunction with the NP-C suspicion index (NP-C SI) for guiding confirmatory laboratory testing in patients with suspected NP-C. Methods: In a prospective observational cohort study, incorporating a retrospective determination of NP-C SI scores, two different diagnostic approaches were applied in two separate groups of unrelated patients from 51 Spanish medical centers (n = 118 in both groups). From Jan 2010 to Apr 2012 (Period 1), patients with =2 clinical signs/symptoms of NP-C were considered ''suspected NP-C'' cases, and NPC1/NPC2 sequencing, plasma chitotriosidase (ChT), CCL18/PARC and sphingomyelinase levels were assessed. Based on findings in Period 1, plasma ChT and CCL18/PARC, and NP-C SI prediction scores were determined in a second group of patients between May 2012 and Apr 2014 (Period 2), and NPC1 and NPC2 were sequenced only in those with elevated ChT and/or elevated CCL18/PARC and/or NP-C SI =70. Filipin staining and 7-ketocholesterol (7-KC) measurements were performed in all patients with NP-C gene mutations, where possible. Results: In total across Periods 1 and 2, 10/236 (4%) patients had a confirmed diagnosis o NP-C based on gene sequencing (5/118 4.2%] in each Period): all of these patients had two causal NPC1 mutations. Single mutant NPC1 alleles were detected in 8/236 (3%) patients, overall. Positive filipin staining results comprised three classical and five variant biochemical phenotypes. No NPC2 mutations were detected. All patients with NPC1 mutations had high ChT activity, high CCL18/PARC concentrations and/or NP-C SI scores =70. Plasma 7-KC was higher than control cut-off values in all patients with two NPC1 mutations, and in the majority of patients with single mutations. Family studies identified three further NP-C patients. Conclusion: This approach may be very useful for laboratories that do not have mass spectrometry facilities and therefore, they cannot use other NP-C biomarkers for diagnosis

    Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)

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    Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1.

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    In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to examine autophagy and related processes, and for reviewers to provide realistic and reasonable critiques of reports that are focused on these processes. These guidelines are not meant to be a dogmatic set of rules, because the appropriateness of any assay largely depends on the question being asked and the system being used. Moreover, no individual assay is perfect for every situation, calling for the use of multiple techniques to properly monitor autophagy in each experimental setting. Finally, several core components of the autophagy machinery have been implicated in distinct autophagic processes (canonical and noncanonical autophagy), implying that genetic approaches to block autophagy should rely on targeting two or more autophagy-related genes that ideally participate in distinct steps of the pathway. Along similar lines, because multiple proteins involved in autophagy also regulate other cellular pathways including apoptosis, not all of them can be used as a specific marker for bona fide autophagic responses. Here, we critically discuss current methods of assessing autophagy and the information they can, or cannot, provide. Our ultimate goal is to encourage intellectual and technical innovation in the field

    Nurses' perceptions of aids and obstacles to the provision of optimal end of life care in ICU

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    Contains fulltext : 172380.pdf (publisher's version ) (Open Access

    Representaciones cartográficas del paisaje singular del olivar en el entorno de la (super)intendencia de las nuevas poblaciones de Sierra Morena. El caso de Baños de la Encina (1752-1797)

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    The unique landscape of the olive grove in Jaen, a space that existed prior to the last third of the 18th century,was cartographically represented between 1792-1797 as one of the consequences of the foundation of the newsettlements of Sierra Morena, in 1767. This leads us to ask ourselves, among other questions, what territories and places, how many trees and what capacities did the spaces dedicated to olive cultivation have before the foundations of Carlos III. The case study of the population and jurisdiction of Baños de la Encina, which was adjacent to the new La Carolina settlements, demonstrates how, with the data obtained from the Ensenada cadastre (1749-1752), we can confirm that there was a high number of plantations, in different parts of the surroundings, which transformed the landscape and contributed to the wealth of the area prior to the agricultural project of the Count of Campomanes and Pablo de Olavide. The methodology used has beenmultidisciplinary, combining various areas of Humanities (History, Geography, Archaeology, Anthropology, etc.) and Sciences (Geomatics, Topography, etc.), and the use of tools such as field studies, Metrology, Toponomastics, GIS. The results are related to the presentation of new experimental theories of knowledge applied to the olive grove: total surface area occupied, location in various places, densities and distances between bushes, crop orientation, etc. The main goal of this analysis is to contribute to the enhancement of spaces of agricultural heritage interest that constitute a sign of identity as well as an added value of the local and provincial level.El paisaje singular del olivar jiennense, espacio existente con anterioridad al último tercio del siglo XVIII, fue representado cartográficamente entre 1792-1797 como una de las consecuencias de la fundación de las nuevas poblaciones de Sierra Morena en 1767. Esto nos lleva a preguntarnos, entre otras cuestiones, qué territorios y parajes, cuántos árboles y de qué capacidades tuvieron los espacios dedicados al cultivo olivarero antes de las fundaciones de Carlos III. El caso de estudio de la población y jurisdicción de Baños de la Encina, que fue aledaña a las nuevas poblaciones carolinas, demuestra cómo, con los datos obtenidos del catastro de Ensenada (1749-1752), podemos confirmar que hubo un elevado número de plantíos, en diferentes parajes de los entornos, que transformaron el paisaje y contribuyeron a la riqueza de la zona con anterioridad al proyecto agrícola del conde de Campomanes y Pablo de Olavide. La metodología empleada ha sido multidisciplinarcombinando diversas áreas de Humanidades (Historia, Geografía, Arqueología, Antropología, etc.) y deCiencias (Geomática, Topografía, etc.), y el uso de herramientas como los estudios de campo, Metrología, Toponomástica, SIG. Los resultados están relacionados con la presentación de nuevas teorías experimentalesdel conocimiento aplicadas al olivar: total de superficie ocupada, localización en diversos parajes, densidadesy distancias entre matas, orientación de los cultivos, etc. El fin último del análisis es contribuir a la puestaen valor de espacios de interés patrimonial agrario que constituyen un signo de identidad, además de unvalor añadido del ámbito local y provincial
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