2,135 research outputs found
Recent Advances in Modeling Mitochondrial Cardiomyopathy Using Human Induced Pluripotent Stem Cells
No full textAround one third of patients with mitochondrial disorders develop a kind of cardiomyopathy. In these cases, severity is quite variable ranging from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. ATP is primarily generated in the mitochondrial respiratory chain via oxidative phosphorylation by utilizing fatty acids and carbohydrates. Genes in both the nuclear and the mitochondrial DNA encode components of this metabolic route and, although mutations in these genes are extremely rare, the risk to develop cardiac symptoms is significantly higher in this patient cohort. Additionally, infants with cardiovascular compromise in mitochondrial deficiency display a worse late survival compared to patients without cardiac symptoms. At this point, the mechanisms behind cardiac disease progression related to mitochondrial gene mutations are poorly understood and current therapies are unable to substantially restore the cardiac performance and to reduce the disease burden. Therefore, new strategies are needed to uncover the pathophysiological mechanisms and to identify new therapeutic options for mitochondrial cardiomyopathies. Here, human induced pluripotent stem cell (iPSC) technology has emerged to provide a suitable patient-specific model system by recapitulating major characteristics of the disease in vitro, as well as to offer a powerful platform for pre-clinical drug development and for the testing of novel therapeutic options. In the present review, we summarize recent advances in iPSC-based disease modeling of mitochondrial cardiomyopathies and explore the patho-mechanistic insights as well as new therapeutic approaches that were uncovered with this experimental platform. Further, we discuss the challenges and limitations of this technology and provide an overview of the latest techniques to promote metabolic and functional maturation of iPSC-derived cardiomyocytes that might be necessary for modeling of mitochondrial disorders.Open-Access-Publikationsfonds 202
Moderata Durant. Connessione tra frammento e città nel progetto d’inserimento del Conservatorio G. Briccialdi nell’ex mercato coperto di Terni
Full text linkLAUREA MAGISTRALE"MODERATA DURANT”. Questa è l’iscrizione che si trova sopra l’architrave dell’ingresso del Conservatorio G. Briccialdi di Terni. La frase latina attesta il destino duraturo delle opere create nel giusto modo e che trovano la giusta misura.
Dunque, la scelta del titolo è intesa a manifestare l’intenzione di identificare quella che è la matrice che permane in una città la cui struttura urbana è stata fortemente alterata nell’ultimo secolo, prima dai bombardamenti della Seconda guerra mondiale e poi dalla sperimentazione di piano di Mario Ridolfi che ha delineato l’impianto della città presente.
Ci si propone quindi di definire il modo e la misura che restituiscano la piazza del Mercato alla città di Terni, tramite un progetto per la nuova sede del conservatorio G. Briccialdi.
La piazza attualmente è un tassello scollegato all’interno del centro cittadino: ricostruita dopo la distruzione della guerra senza tenere conto della morfologia del tessuto medievale circostante e sottostante, si impone come una cesura significativa nella continuità dell’edificato e dello spazio pubblico cittadino. Manifesto della modernità veloce degli anni del dopoguerra, con la chiusura del mercato coperto ha perso anche la sua vocazione di luogo pubblico aperto alla cittadinanza.
La struttura dell’ex-mercato è ora un oggetto alieno in una piazza estranea al tessuto circostante e dunque l’insieme presenta uno stato di degrado e di abbandono inusuale per una parte così significativa del centro storico di una città di medie dimensioni.
Contrariamente alle varie proposte che intendono privatizzare la struttura dell’ex mercato coperto, rendendolo un centro commerciale, la tesi vuole proporre l’intervento pubblico come motore per la riattivazione del tessuto architettonico e sociale.
Si è individuato così il conservatorio G. Briccialdi, attualmente collocato in una struttura carente di spazi e servizi, come nuovo uso in grado di riattivare tutto il contesto. Il progetto della scuola è integrato dalla creazione di un auditorium in grado di diventare polo attrattore di una città in cerca di una nuova vocazione rispetto a quella industriale, così forte in passato ma ora in decisa discussione.
L’edificio pubblico risponde a queste esigenze, manifesta la volontà di riappropriarsi dello spazio circostante rendendolo effettivamente una piazza urbana e scavando si riallaccia alla memoria della città storica dimenticata e a lungo trascurata.
Il progetto diventa quindi uno strumento di reinterpretazione del luogo che si pone come fine il reinserimento di un frammento all'interno del sistema della città analizzata come processo di stratificazione, tramite un'operaione che trova il suo fondamento nell'interferenza di tracce appartenenti ad epoche diverse. . Lo scavo ha il ruolo di rendere manifesta questa interferenza, creando una cerniera tra il sistema dell’auditorium, del conservatorio e dello spazio pubblico: la memoria storica diventa così capace di assumere un ruolo decisivo tanto per il progetto quanto per la città, proponendo una maniera più consapevole e attenta al luogo di interpretare il progetto e le trasformazioni urbane."MODERATA DURANT". This is the inscription found above the architrave of the entrance to the G. Briccialdi Conservatory in Terni. The Latin phrase attests to the enduring destiny of the works created in the right way and that find the right measure.
Therefore, the choice of the title is aimed to show what is the matrix that remains in a city whose urban structure has been strongly altered in the last century, first by the World War II bombings and then by the experimentation of the urban city plan by Mario Ridolfi, who outlined the present city structure.
We therefore want to define the way and the extent by which the Piazza del Mercato goes back to the city of Terni, through a project for the new Headquarters of the G. Briccialdi conservatory.
The square appears currently an unconnected block inside the city center: rebuilt after the war without taking into account the morphology of the surrounding and underlying medieval fabric, it imposes itself as a significant caesura in the continuity of the buildings and public space. Manifesto of the fast modernity of the post-war years, with the closure of the covered market it has also lost its vocation as a public place open to citizens.
The structure of the previous market is now an alien object in a square unrelated to the surrounding fabric and therefore the whole presents a state of degradation and unusual abandon for such a significant part of the historical center of a medium-sized city.
Unlike the various proposals that intend to privatize the structure of the former covered market making it a mall, this study aims to propose public intervention as an engine for the reactivation of the architectural and social fabric.
Thus the G. Briccialdi conservatory, currently located in a structure lacking both space and services, is identified as a new function capable of reactivating the whole context. The school project is complemented by the creation of an auditorium capable of becoming a centre of attraction for a city in search of a new vocation other than the industrial one, so marked in the past but now strongly questioned.
The public building responds to these needs; it expresses the desire to reclaim the surrounding space, actually making it a urban square and in its depth it finds again the memory of the ancient city ,so long neglected.
This project thus becomes a tool for reinterpretation of the place, aimed to re-inserting a fragment within the city system considered as a stratification process, with traces belonging to different ages. The excavation has the goal of making this interference manifest, creating a link between the auditorium, the conservatory and the public space: the historical memory thus becomes capable of assuming a decisive role both for the project and for the city, proposing a more conscious and attentive approach to the project and the urban transformations
Refuse recovered biomass fuel from municipal solid waste. A life cycle assessment
No full textWaste disposal is a controversial issue in many European countries: concerns about potential health effects and land value loss as well as the fulfillment of the European Landfill Directive and Waste Framework Directive have significantly changed the way waste should be managed. An appropriate management of municipal solid waste (MSW) may allow a significant enhancement of efficiency in resources use, by recovering both energy and materials from waste, otherwise landfilled, thus replacing fossil fuels and virgin materials with renewable sources. Separation and recovery of the biodegradable fraction of municipal solid waste is encouraged as a mean to produce bioenergy. Therefore, if not source segregated, innovative waste refining technologies may provide potential solutions for separation of organic fraction and improved energy and materials recovery. This paper presents a comprehensive system study of a recently developed technology aimed to improve the MSW management in order to decrease the demand for new landfill space and, at the same time, contribute to the urban energy needs. As part of a wider Life Plus Project entitled MARSS (Material Advanced Recovery Sustainable Systems), funded by European Community in 2012, the environmental assessment of an innovative and enhanced mechanical and biological treatment (MBT) demo plant installed in Mertesdorf (Germany) was performed by means of the SimaPro 8.0.5 LCA software, utilizing ReCiPe (H) Midpoint method for the impact assessment. The plant under study is designed to concentrate the biodegradable part of MSW in the <40 mm fraction, through a series of refining and recovery steps, to remove contaminants and obtain a suitable biomass fuel with a final marketable quality fulfilling the requirements for biomass power plants to generate urban decentralized production of heat and power (CHP). This study aims at understanding if and to what extent the MBT-MARSS plant is environmentally sound, by investigating environmental costs and benefits of replacing MSW landfilling and waste-to-energy disposal by means of boosted separation of biomass for energy generation in CHPs and other recoverable fractions (metals, plastic). Steps and/or components that can be further improved are also assessed. Sensitivity of impacts to assumptions regarding the source of replaced electricity was also tested. Results not only emphasize the novelty of a promising new technology, but also the extent of benefits that can be achieved depending on the actual power generation technique that is replaced by means of the energy recovered in the process. The quantitative evaluation of the MARSS technology shows that appropriate design and management of the MBT plant lead to substantial reduction of environmental impacts as well as material and energy resource savings, thus putting forward a technical solution suitable for those cities/countries where other solutions are still lacking or inappropriate or unfeasible
El Tlacuache Núm. 221 (2006). 221 Año 7 (2006) agosto. El Tlacuache
No full textProyecto Chalcatzingo por Mario Córdova Tello. - Un pueblo olmeca en Tequesquitengo por G. Manuel Barragán Dorantes y Giselle Canto Aguilar. - Curso de Verano Infantil del Jardín Etnobotánico por Leticia Gabriela Avalos Becerril
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