10.1 El conflicto por el régimen. Historia oficial y políticas de memoria en España (1978-2013)

Anàlisi de la història oficial i de les polítiques de memòria a Espanya (1978-2013)Historia oficial y políticas de memoria en España (1978-2013

El cemento natural en el Madrid del S. XIX

El cemento natural fue patentado en Inglaterra en 1796, pero no llegó a España hasta 1835, aunque todavía se discute dónde comenzó la producción nacional, ya que surgió casi simultáneamente en el País Vasco (Zumaya y Rezola) y en Cataluña (San Celoní y San Juan de las Abadesas). Desde entonces fue ampliamente utilizado en la ornamentación arquitectónica durante el siglo XIX y principios del siglo XX en Madrid. Con su llegada reemplazó materiales tradicionales que se utilizaban anteriormente, como las cales aéreas e hidráulicas, y el yeso. Sin embargo, su uso no se alargó en el tiempo, pues pronto fue sustituido por el cemento Portland. En la actualidad, lo que queda de el son cientos de " testimonios de piedra" que hay que conservar y reparar ocasionalmente de la mejor manera posible. Las propiedades finales del cemento natural dependían en gran medida de las materias primas utilizadas y su temperatura de obtención y en general, se caracterizaba por un fraguado rápido (aproximadamente de 15 minutos), una buena resistencia y un agradable color ocre. Esta comunicación muestra las características de las fachadas madrileñas del S.XIX recubiertas con morteros de cemento natural, su estado de conservación y deterioro, así como los daños producidos como resultado de intervenciones desafortunadas en las que han empleado materiales no compatibles con este cemento

Powertrain modelling for engine stop-start dynamics and control of micro/mild hybrid construction machines

Engine stop-start control is considered as the key technology for micro/mild hybridisation of vehicles and machines. To utilize this concept, especially for construction machines, the engine is desired to be started in such a way that the operator discomfort can be minimized. To address this issue, this paper aims to develop a simple powertrain modelling approach for engine stop-start dynamic analysis and an advanced engine start control scheme newly applicable for micro/mild hybrid construction machines. First, a powertrain model of a generic construction machine is mathematically developed in a general form which allows to investigate the transient responses of the system during the engine cranking process. Second, a simple parameterisation procedure with a minimum set of data required to characterise the dynamic model is presented. Third, a model- based adaptive controller is designed for the starter to crank the engine quickly and smoothly without the need of fuel injection while the critical problems of machine noise, vibration and harshness can be eliminated. Finally, the advantages and effectiveness of the proposed modelling and control approaches have been validated through numerical simulations. The results imply that with the limited data set for training, the developed model works better than a high fidelity model built in AMESim while the adaptive controller can guarantee the desired cranking performance

Powertrain modelling and engine start control of construction machines

This paper aims to develop an engine start control approach for a micro/mild hybrid machine for a capable of cranking the engine without injection. First, the powertrain is physically modelled using a co-simulation platform. Second, experiment data of the traditional machine is acquired to optimize the model. Third, a model-based adaptive controller is designed for the starter to crank the engine quickly and smoothly to minimize the operator discomfort. The effectiveness of the proposed approach is validated through numerical simulations with the established model

Challenges of micro/mild hybridisation for construction machinery and applicability in UK

In recent years, micro/mild hybridisation (MMH) is known as a feasible solution for powertrain development with high fuel efficiency, less energy use and emission and, especially, low cost and simple installation. This paper focuses on the challenges of MMH for construction machines and then, pays attention to its applicability to UK construction machinery. First, hybrid electric configurations are briefly reviewed; and technological challenges towards MMH in construction sector are clearly stated. Second, the current development of construction machinery in UK is analysed to point out the potential for MMH implementation. Thousands of machines manufactured in UK have been sampled for the further study. Third, a methodology for big data capturing, compression and mining is provided for a capable of managing and analysing effectively performances of various construction machine types. By using this method, 96% of data memory can be reduced to store the huge machine data without lacking the necessary information. Forth, an advanced decision tool is built using a fuzzy cognitive map based on the big data mining and knowledge from experts to enables users to define a target machine for MMH utilization. The numerical study with this tool on the sampled machines has been done and finally realized that one class of heavy excavators is the most suitable to apply MMH technology

IGF-1 receptor antagonism inhibits autophagy

Inhibition of the insulin/insulin-like growth factor signalling pathway increases lifespan and protects against neurodegeneration in model organisms, and has been considered as a potential therapeutic target. This pathway is upstream of mTORC1, a negative regulator of autophagy. Thus, we expected autophagy to be activated by insulin-like growth factor-1 (IGF-1) inhibition, which could account for many of its beneficial effects. Paradoxically, we found that IGF-1 inhibition attenuates autophagosome formation. The reduced amount of autophagosomes present in IGF-1R depleted cells can be, at least in part, explained by a reduced formation of autophagosomal precursors at the plasma membrane. In particular, IGF-1R depletion inhibits mTORC2, which, in turn, reduces the activity of protein kinase C (PKCa/b). This perturbs the actin cytoskeleton dynamics and decreases the rate of clathrin-dependent endocytosis, which impacts autophagosome precursor formation. Finally, with important implications for human diseases, we demonstrate that pharmacological inhibition of the IGF-1R signalling cascade reduces autophagy also in zebrafish and mice models. The novel link we describe here has important consequences for the interpretation of genetic experiments in mammalian systems and for evaluating the potential of targeting the IGF-1R receptor or modulating its signalling through the downstream pathway for therapeutic purposes under clinically relevant conditions, such as neurodegenerative diseases, where autophagy stimulation is considered beneficial.This is the version of the manuscript that was first published on line. The final version can be found published in Human Molecular Genetics by OUP here: http://hmg.oxfordjournals.org/content/22/22/4528.full.pdf+html

Deficiency of the zinc finger protein ZFP106 causes motor and sensory neurodegeneration

Acknowledgements We are indebted to Jim Humphries, JennyCorrigan, LizDarley, Elizabeth Joynson, Natalie Walters, Sara Wells and the whole necropsy, histology, genotyping and MLC ward 6 teams at MRC Harwell for excellent technical assistance. We thank the staff of the WTSI Illumina Bespoke Team for the RNA-seq data, the Sanger Mouse Genetics Project for the initial mouse characterization and Dr David Adams for critical reading of the manuscript. We also thank KOMP for the mouse embryonic stem cells carrying the knockout first promoter-less allele (tm1a(KOMP)Wtsi) within Zfp016. Conflict of Interest statement. None declared. Funding This work was funded by the UK Medical Research Council (MRC) to A.A.-A. and a Motor Neurone Disease Association (MNDA) project grant to A.A.-A. and EMCF. D.L.H.B. is a Wellcome Trust Senior Clinical Scientist Fellow and P.F. is a MRC/MNDA Lady Edith Wolfson Clinician Scientist Fellow. Funding to pay the Open Access publication charges for this article was provided by the MRC grant number: MC_UP_A390_1106.Peer reviewedPublisher PD

A genetic modifier suggests that endurance exercise exacerbates Huntington's disease.

Polyglutamine expansions in the huntingtin gene cause Huntington's disease (HD). Huntingtin is ubiquitously expressed, leading to pathological alterations also in peripheral organs. Variations in the length of the polyglutamine tract explain up to 70% of the age-at-onset variance, with the rest of the variance attributed to genetic and environmental modifiers. To identify novel disease modifiers, we performed an unbiased mutagenesis screen on an HD mouse model, identifying a mutation in the skeletal muscle voltage-gated sodium channel (Scn4a, termed 'draggen' mutation) as a novel disease enhancer. Double mutant mice (HD; Scn4aDgn/+) had decreased survival, weight loss and muscle atrophy. Expression patterns show that the main tissue affected is skeletal muscle. Intriguingly, muscles from HD; Scn4aDgn/+ mice showed adaptive changes similar to those found in endurance exercise, including AMPK activation, fibre type switching and upregulation of mitochondrial biogenesis. Therefore, we evaluated the effects of endurance training on HD mice. Crucially, this training regime also led to detrimental effects on HD mice. Overall, these results reveal a novel role for skeletal muscle in modulating systemic HD pathogenesis, suggesting that some forms of physical exercise could be deleterious in neurodegeneration