Metabolic Alterations in Cardiomyocytes of Patients with Duchenne and Becker Muscular Dystrophies

Duchenne and Becker muscular dystrophies (DMD/BMD) result in progressive weakness of skeletal and cardiac muscles due to the deficiency of functional dystrophin. Respiratory failure is a leading cause of mortality in DMD patients; however, improved management of the respiratory symptoms have increased patients’ life expectancy, thereby also increasing the clinical relevance of heart disease. In fact, the prevalence of cardiomyopathy, which significantly contributes to mortality in DMD patients, increases with age and disease progression, so that over 95% of adult patients has cardiomyopathy signs. We here review the current literature featuring the metabolic alterations observed in the dystrophic heart of the mdx mouse, i.e., the best-studied animal model of the disease, and discuss their pathophysiological role in the DMD heart. It is well assessed that dystrophin deficiency is associated with pathological alterations of lipid metabolism, intracellular calcium levels, neuronal nitric oxide (NO) synthase localization, and NO and reactive oxygen species production. These metabolic stressors contribute to impair the function of the cardiac mitochondrial bulk, which has a relevant pathophysiological role in the development of cardiomyopathy. In fact, mitochondrial dysfunction becomes more severe as the dystrophic process progresses, thereby indicating it may be both the cause and the consequence of the dystrophic process in the DMD heart

A study on the cement-based decorative materials in the San Fedele Church in Milan

Cement-based materials have been used since the 19th century for different decorative purposes, and a high levels of expertise has often reached in reproducing or restoring even quite elaborated stoneworks. An important example is the application of cement-based decorative materials on the façades of the San Fedele church in Milan. The church, built in the 16th century and characterized by the presence of pinkish-yellowish Angera stone on the façades, was subjected, especially in the 20th century, to several restoration works. Damaged decorative elements of the façades as well as portions of its structural elements were replaced or covered in the last century by â\u80\u9cstone imitating renderâ\u80\u9d, made with cementitious materials which imitate the original Angera stone. In this study, several samples of cement-based decorative materials, collected from different elements of the external façades of the Church, were characterized by several analytic techniques (thermogravimetric analysis, X-ray diffraction, scanning electron microscopy and IR analysis), in order to investigate both their microstructure and composition, how the chromatic aspect of the cementitious materials were obtained and their conservation state and to provide useful information for the possible reproduction of materials with comparable appearance to be used in a further restoration project. Results showed that the cement-based materials and decorations were obtained by the application of different layers of renders; in particular, the colour and texture of finishing layer were achieved by blending the binder with fine dolomite particles, probably obtained by grinding the Angera stone. This technique not only allowed an amazing reproduction of the original stone, but also resulted in a durable protection, since the cement-based decorative materials did not show any significant degradation phenomena in the polluted environment of the centre of Milan

Corrosion behaviour of metal inserts in simulated ancient masonry mortars

Corrosion of metal inserts may be detrimental to the durability of masonry. The paper discusses corrosion of carbon steel, stainless steel and titanium embedded in aerial and hydraulic mortars simulating those of ancient walls. The effects of temperature (5-40°C), moisture (65-95% RH and water suction) and electrical resistivity of mortars on the corrosion of metal inserts are discussed. Results showed that the corrosion rate of embedded steel, regardless of the mortar composition, is negligible in mortars exposed to 65-80% RH (even at 40°C) and modest even at 95% RH, while high values were obtained in the presence of water uptake

The challenge of the performance-based approach for the design of reinforced concrete structures in chloride bearing environment

The performance-based approach, published by the International Federation for Structural Concrete (fib), was applied for the design of a RC element in a marine environment, with corrosion resistant reinforcement, to analyse the potentiality of the model as well as the possible reasons which limit its use. Results showed that the fib model allows to compare different solutions and to consider the benefits connected with the use of preventative measures. However the definition of reliable values for some input parameters, as the critical chloride threshold for corrosion resistant reinforcement, is demanded to the designer and this aspect clearly limits a widespread use

Corrosion of Steel in Concrete and Its Prevention in Aggressive Chloride-Bearing Environments

This keynote paper deals with the durability of reinforced concrete (RC) structures exposed to aggressive environments characterized by high concentration of chloride ions, namely, marine environments or the use of de-icing salts. The mechanism of chloride-induced corrosion of steel in concrete is introduced, and its influence on the service life of RC structures is analyzed. Factors affecting the time to corrosion initiation are described with regard to both concrete properties and environmental exposure conditions. Design approaches available for achieving durability targets associated with the design service life are analyzed, focusing on studies carried out by the authors in recent years at the mCD Concrete Durability lab of Politecnico di Milano, which were aimed at improving the protection provided to the steel bars by the concrete cover, investigating the advantages of using corrosion-resistant stainless steel bars and developing the electrochemical technique of cathodic prevention

Prueba utilizada en investigaciones de prácticas anticompetitivas en El Salvador: criterios de la Sala de lo Contencioso Administrativa 2006-2019

The Competition Law of El Salvador of 2006 prohibits cartels between businessmen or economic operators, but since they are illegal agreements, generally carried out without leaving a material evidence trail, there is difficulty in proving their realization. This has generated multiple challenges related to the lack of forcefulness to justify the sanctions imposed, in the opinion of the affected companies. Many sanctions imposed by the Board of Directors of the Superintendency of Competition have been challenged in Contentious-Administrative proceedings, as it was the only jurisdiction before which appeals could be made during the period covered by this investigation. The methodology used in this article consisted of the analysis of all the resolutions in cases of cartels sanctioned by the competition authority, to extract the evidence that has allowed convicting; and in a second place, to examine the rulings of the Contentious Chamber of the Supreme Court of Justice of El Salvador to determine if they have confirmed the legality of the decisions of the competition authority or if they have established their illegality. The most relevant finding of this study, which can help economic operators to guide their actions within the framework of the Competition Law, is that the legality of all sanctions imposed in cartel cases has been confirmed.La Ley de Competencia de El Salvador del 2006, prohíbe los carteles entre empresarios u operadores económicos, pero como se trata de acuerdos ilegales, realizados generalmente sin dejar un rastro probatorio material, existe dificultad para probar su realización. Esto ha generado múltiples impugnaciones relacionadas con la falta de contundencia para justificar las sanciones impuestas, en la opinión de las empresas afectadas. Muchas sanciones impuestas por el Consejo Directivo de la Superintendencia de Competencia han sido impugnadas en sede Contencioso Administrativa, por ser la única jurisdicción ante la cual se podía recurrir durante el periodo cubierto por esta investigación. La metodología empleada en el presente artículo, consistió en el análisis de la totalidad de las resoluciones en casos de carteles sancionados por la autoridad de competencia, para extraer los elementos probatorios que han permitido condenar; y en un segundo lugar, examinar los pronunciamientos de la Sala de lo Contencioso de la Corte Suprema de Justicia de El Salvador para determinar si han confirmado la legalidad de las decisiones de la autoridad de competencia o si han establecido su ilegalidad. El hallazgo más relevante de este estudio y que puede servir para que los operadores económicos orienten sus actuaciones en el marco de la Ley de Competencia, es que la legalidad de todas las sanciones impuestas en casos de carteles ha sido confirmada

Sustainable Concrete with Seawater and Corrosion Resistant Reinforcement: Results of Monitoring of Corrosion Behaviour

The use of seawater for mixing concrete for reinforced concrete structures is prohibited, since it can promote steel corrosion. However, the use of seawater would contribute to decrease the environmental impact of concrete, in particular in those regions of the world where potable water is a precious resource. The project SEACON-INFRAVATION between University of Miami and Politecnico di Milano, with various industrial partners, aims at investigating the use of seawater for the construction of sustainable and durable reinforced concrete structures and infrastructures. Within the project, that included a vast campaign of laboratory tests, also two demo projects – one in Italy and another one in the US – were designed and executed with the aim of testing the technology on-site and allowing long-term monitoring of the durability behavior. In Italy, a reinforced concrete culvert was built next to A1 motorway, close to Piacenza. The culvert collects the waters coming from the roadway that, during winter season, is subjected to de-icing salts; in addition, it is unsheltered from the rain and exposed to wetting and drying cycles. The culvert is divided into different segments, and each segment is representative of a given scenario in terms of type of concrete and type of reinforcement. Besides a reference segment, with carbon steel and chloride-free concrete, some segments were built using seawater concrete in combination with corrosion-resistant reinforcement. Three types of corrosion-resistant reinforcement were considered: an austenitic grade of stainless steel (1.4311), a duplex grade of stainless steel (1.4362) and GFRP. The corrosion conditions of the metallic reinforcements are monitored by means of potential measurements. The electrical resistivity of concrete is also measured in time, and the evolution of carbonation and chloride penetration are periodically analysed on concrete cores taken from the culvert. This note presents the results that have been obtained during more than one year of monitoring of the corrosion conditions of the various types of reinforcement embedded in seawater concrete and compares them with results obtained in the laboratory

3D survey technologies: Investigations on accuracy and usability in archaeology. The case study of the new "Municipio" underground station in Naples

Advanced 3D survey technologies, such as Digital Photogrammetry (imaged based) and Laser Scanner, are nowadays widely used in Cultural Heritage and Archaeological fields. The present paper describes the investigations realized by the Laboratory Hesutech of the Polytechnic of Milan in cooperation with the Superintendence Archaeology Campania in order to examine the potentiality of Image Based Modeling (IBM) systems applied to the archaeological field for advanced documentation purposes. Besides the 3D model production workflow in an uncommon excavation environment, a special consideration about the reached accuracy will be discussed. In the first part of the research, a comparison between photogrammetric camera parameters obtained with IBM systems and the ones provided with the calibration certificate by the manufacturer of the camera is performed. In the second part of the research, the operational phases of the application of such advanced 3D survey technologies are shown. The test field is the archaeological excavation area for the construction of the new "Municipio" underground station in Naples. Due to its position in one of the historical area of the city, its construction coexists with the archaeological excavations and it is strictly tied to their evolution. In such conditions, the need to reduce as much as possible the time to build the public infrastructure is a very relevant feature together with the ability to produce accurate documentation of what is considered archaeologically important

Molecular analysis of Duchenne/Becker muscular dystrophy

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Can seawater be used as mixing water for durable and sustainable RC structures?

Nowadays the use of chloride-contaminated raw materials is prohibited for reinforced concrete (RC) structures. Beside possible effects on the early stages of cement hydration and the long-term development of strength, the primary reason is corrosion of black steel reinforcement. In fact, it is well known that chlorides, destroying the passivation film, make steel susceptible to pitting corrosion. Thus, design standards worldwide aim at limiting the use of chloride-contaminated materials. However, the use of chloride-contaminated raw materials for the production of concrete would be advantageous since it would allow saving natural resources, such as fresh water, leading to enhanced environmental sustainability. In the framework of a research project financed by the Infravation Program (Advanced systems, materials and techniques for next generation infrastructure), an experimental study is undertaken aiming at demonstrating the safe utilization of seawater and salt-contaminated aggregates (natural or recycled) for a sustainable concrete production when combined with non-corrosive reinforcement to construct durable and economical concrete infrastructures. This paper focuses on a preliminary evaluation of the possibility of replacing fresh water with seawater when combined with different types of stainless steel reinforcement. Through a performance-based approach, RC elements made with fresh water and seawater, different constituents and stainless steel bars were simulated under exposure to different marine environments to define possible materials combinations able to guarantee a target design service life