Neonatal gene transfer leads to widespread correction of pathology in a murine model of lysosomal storage disease

For many inborn errors of metabolism, early treatment is critical to prevent long-term developmental sequelae. We have used a gene-therapy approach to demonstrate this concept in a murine model of mucopolysaccharidosis type VII (MPS VII). Newborn MPS VII mice received a single intravenous injection with 5.4 × 106 infectious units of recombinant adeno-associated virus encoding the human β-glucuronidase (GUSB) cDNA. Therapeutic levels of GUSB expression were achieved by 1 week of age in liver, heart, lung, spleen, kidney, brain, and retina. GUSB expression persisted in most organs for the 16-week duration of the study at levels sufficient to either reduce or prevent completely lysosomal storage. Of particular significance, neurons, microglia, and meninges of the central nervous system were virtually cleared of disease. In addition, neonatal treatment of MPS VII mice provided access to the central nervous system via an intravenous route, avoiding a more invasive procedure later in life. These data suggest that gene transfer mediated by adeno-associated virus can achieve therapeutically relevant levels of enzyme very early in life and that the rapid growth and differentiation of tissues does not limit long-term expression.

Interaction between like-charged colloidal spheres in electrolyte solutions

How colloidal particles interact with each other is one of the key issues that determines our ability to interpret experimental results for phase transitions in colloidal dispersions and our ability to apply colloid science to various industrial processes. The long-accepted theories for answering this question have been challenged by results from recent experiments. Herein we show from Monte-Carlo simulations that there is a short-range attractive force between identical macroions in electrolyte solutions containing divalent counterions. Complementing some recent and related results by others, we present strong evidence of attraction between a pair of spherical macroions in the presence of added salt ions for the conditions where the interacting macroion pair is not affected by any other macroions that may be in the solution. This attractive force follows from the internal-energy contribution of counterion mediation. Contrary to conventional expectations, for charged macroions in an electrolyte solution, the entropic force is repulsive at most solution conditions because of localization of small ions in the vicinity of macroions. Both Derjaguin–Landau–Verwey–Overbeek theory and Sogami–Ise theory fail to describe the attractive interactions found in our simulations; the former predicts only repulsive interaction and the latter predicts a long-range attraction that is too weak and occurs at macroion separations that are too large. Our simulations provide fundamental “data” toward an improved theory for the potential of mean force as required for optimum design of new materials including those containing nanoparticles.

Functional and structural mapping of human cerebral cortex: Solutions are in the surfaces

The human cerebral cortex is notorious for the depth and irregularity of its convolutions and for its variability from one individual to the next. These complexities of cortical geography have been a chronic impediment to studies of functional specialization in the cortex. In this report, we discuss ways to compensate for the convolutions by using a combination of strategies whose common denominator involves explicit reconstructions of the cortical surface. Surface-based visualization involves reconstructing cortical surfaces and displaying them, along with associated experimental data, in various complementary formats (including three-dimensional native configurations, two-dimensional slices, extensively smoothed surfaces, ellipsoidal representations, and cortical flat maps). Generating these representations for the cortex of the Visible Man leads to a surface-based atlas that has important advantages over conventional stereotaxic atlases as a substrate for displaying and analyzing large amounts of experimental data. We illustrate this by showing the relationship between functionally specialized regions and topographically organized areas in human visual cortex. Surface-based warping allows data to be mapped from individual hemispheres to a surface-based atlas while respecting surface topology, improving registration of identifiable landmarks, and minimizing unwanted distortions. Surface-based warping also can aid in comparisons between species, which we illustrate by warping a macaque flat map to match the shape of a human flat map. Collectively, these approaches will allow more refined analyses of commonalities as well as individual differences in the functional organization of primate cerebral cortex.

Characterization of SU1 Isoamylase, a Determinant of Storage Starch Structure in Maize1

Function of the maize (Zea mays) gene sugary1 (su1) is required for normal starch biosynthesis in endosperm. Homozygous su1- mutant endosperms accumulate a highly branched polysaccharide, phytoglycogen, at the expense of the normal branched component of starch, amylopectin. These data suggest that both branched polysaccharides share a common precursor, and that the product of the su1 gene, designated SU1, participates in kernel starch biosynthesis. SU1 is similar in sequence to α-(1→6) glucan hydrolases (starch-debranching enzymes [DBEs]). Specific antibodies were produced and used to demonstrate that SU1 is a 79-kD protein that accumulates in endosperm coincident with the time of starch biosynthesis. Nearly full-length SU1 was expressed in Escherichia coli and purified to apparent homogeneity. Two biochemical assays confirmed that SU1 hydrolyzes α-(1→6) linkages in branched polysaccharides. Determination of the specific activity of SU1 toward various substrates enabled its classification as an isoamylase. Previous studies had shown, however, that su1- mutant endosperms are deficient in a different type of DBE, a pullulanase (or R enzyme). Immunoblot analyses revealed that both SU1 and a protein detected by antibodies specific for the rice (Oryza sativa) R enzyme are missing from su1- mutant kernels. These data support the hypothesis that DBEs are directly involved in starch biosynthesis.

Lipoxygenase-catalyzed oxygenation of storage lipids is implicated in lipid mobilization during germination.

The etiolated germination process of oilseed plants is characterized by the mobilization of storage lipids, which serve as a major carbon source for the seedling. We found that during early stages of germination in cucumber, a lipoxygenase (linoleate: oxygen oxidoreductase, EC 1.13.11.12) form is induced that is capable of oxygenating the esterified fatty acids located in the lipid-storage organelles, the so-called lipid bodies. Large amounts of esterified (13S)-hydroxy-(9Z,11E)-octadecadienoic acid were detected in the lipid bodies, whereas only traces of other oxygenated fatty acid isomers were found. This specific product pattern confirms the in vivo action of this lipoxygenase form during germination. Lipid fractionation studies of lipid bodies indicated the presence of lipoxygenase products both in the storage triacylglycerols and, to a higher extent, in the phospholipids surrounding the lipid stores as a monolayer. The degree of oxygenation of the storage lipids increased drastically during the time course of germination. We show that oxygenated fatty acids are preferentially cleaved from the lipid bodies and are subsequently released into the cytoplasm. We suggest that they may serve as substrate for beta-oxidation. These data suggest that during the etiolated germination, a lipoxygenase initiates the mobilization of storage lipids. The possible mechanisms of this implication are discussed.

Joint interpretation of magnetotelluric, seismic, and well-log data in Hontomín (Spain)

Acknowledgements. This work is dedicated to the memory of Andrés Pérez-Estaún, brilliant scientist, colleague, and friend. The authors sincerely thank Ian Ferguson and an anonymous reviewer for their useful comments on the manuscript. Xènia Ogaya is currently supported in the Dublin Institute for Advanced Studies by a Science Foundation Ireland grant IRECCSEM (SFI grant 12/IP/1313). Juan Alcalde is funded by NERC grant NE/M007251/1, on interpretational uncertainty. Juanjo Ledo, Pilar Queralt and Alex Marcuello thank Ministerio de Economía y Competitividad and EU Feder Funds through grant CGL2014- 54118-C2-1-R. Funding for this Project has been partially provided by the Spanish Ministry of Industry, Tourism and Trade, through the CIUDEN-CSIC-Inst. Jaume Almera agreement (ALM-09-027: Characterization, Development and Validation of Seismic Techniques applied to CO2 Geological Storage Sites), the CIUDEN-Fundació Bosch i Gimpera agreement (ALM-09-009 Development and Adaptation of Electromagnetic techniques: Characterisation of Storage Sites) and the project PIERCO2 (Progress In Electromagnetic Research for CO2 geological reservoirs CGL2009-07604). The CIUDEN project is co-financed by the European Union through the Technological Development Plant of Compostilla OXYCFB300 Project (European Energy Programme for Recovery).

Mapping soil organic carbon storage in deep soil horizons of Amazonian Podzols

The Podzols of the world are divided into intra-zonal and zonal according to then location. Zonal Podzols are typical for boreal and taiga zone associated to climate conditions. Intra-zonal podzols are not necessarily limited by climate and are typical for mineral poor substrates. The Intra-zonal Podzols of the Brazilian Amazon cover important surfaces of the upper Amazon basin. Their formation is attributed to perched groundwater associated to organic matter and metals accumulations in reducing/acidic environments. Podzols have a great capacity of storing important amounts of soil organic carbon in deep thick spodic horizons (Bh), in soil depths ranging from 1.5 to 5m. Previous research concerning the soil carbon stock in Amazon soils have not taken into account the deep carbon stock (below 1 m soil depth) of Podzols. Given this, the main goal of this research was to quantify and to map the soil organic carbon stock in the region of Rio Negro basin, considering the carbon stored in the first soil meter as well as the carbon stored in deep soil horizons up to 3m. The amount of soil organic carbon stored in soils of Rio Negro basin was evaluated in different map scales, from local surveys, to the scale of the basin. High spatial and spectral resolution remote sensing images were necessary in order to map the soil types of the studied areas and to estimate the soil carbon stock in local and regional scale. Therefore, a multi-sensor analysis was applied with the aim of generating a series of biophysical attributes that can be indirectly related to lateral variation of soil types. The soil organic carbon stock was also estimated for the area of the Brazilian portion of the Rio Negro basin, based on geostatistical analysis (multiple regression kriging), remote sensing images and legacy data. We observed that Podzols store an average carbon stock of 18 kg C m-2 on the first soil meter. Similar amount was observed in adjacent soils (mainly Ferralsols and Acrisols) with an average carbon stock of 15 kg C m-2. However if we take into account a 3 m soil depth, the amount of carbon stored in Podzols is significantly higher with values ranging from 55 kg C m-2 to 82 kg C m-2, which is higher than the one stored in adjacent soils (18 kg C m-2 to 25 kg C m-2). Given this, the amount of carbon stored in deep soil horizons of Podzols should be considered as an important carbon reservoir, face a scenario of global climate change

A robust and fast method for 6DoF motion estimation from generalized 3D data

Nowadays, there is an increasing number of robotic applications that need to act in real three-dimensional (3D) scenarios. In this paper we present a new mobile robotics orientated 3D registration method that improves previous Iterative Closest Points based solutions both in speed and accuracy. As an initial step, we perform a low cost computational method to obtain descriptions for 3D scenes planar surfaces. Then, from these descriptions we apply a force system in order to compute accurately and efficiently a six degrees of freedom egomotion. We describe the basis of our approach and demonstrate its validity with several experiments using different kinds of 3D sensors and different 3D real environments.

La comunicación de la innovación de las agencias de medios. Big data en las webs propias

Las agencias de medios constituyen uno de los sectores publicitarios en el que la aplicación de big data proporciona soluciones innovadoras que debieran ser comunicadas a través, entre otros canales, de sus webs corporativas. Con el propósito de facilitar tal comunicación, las webs deben estar preparadas para ser consultadas por varios dispositivos. Así, este estudio pretende averiguar su uso para comunicar innovaciones relacionadas con big data, cuidando el responsive design, de las 20 primeras agencias de medios españolas, y reflexiona sobre las debilidades, amenazas, fortalezas y oportunidades de tal innovación.

Smart Monitoring Embedded Service for Energy-Efficient and Sustainable Management in Data Centers

Information technologies (IT) currently represent 2% of CO2 emissions. In recent years, a wide variety of IT solutions have been proposed, focused on increasing the energy efficiency of network data centers. Monitoring is one of the fundamental pillars of these systems, providing the information necessary for adequate decision making. However, today’s monitoring systems (MSs) are partial, specific and highly coupled solutions. This study proposes a model for monitoring data centers that serves as a basis for energy saving systems, offered as a value-added service embedded in a device with low cost and power consumption. The proposal is general in nature, comprehensive, scalable and focused on heterogeneous environments, and it allows quick adaptation to the needs of changing and dynamic environments. Further, a prototype of the system has been implemented in several devices, which has allowed validation of the proposal in addition to identification of the minimum hardware profile required to support the model.

Novas tendências, o big data: deteção de fraudes e transações no comércio local: SAF-T com tecnologia big data

Dissertação apresentada à Escola Superior de Tecnologia do Instituto Politécnico de Castelo Branco para cumprimento dos requisitos necessários à obtenção do grau de Mestre em Desenvolvimento de Software e Sistemas Interactivos, realizada sob a orientação científica da categoria profissional do orientador Doutor Eurico Ribeiro Lopes, do Instituto Politécnico de Castelo Branco.

P.A.V.E.S. : Post-Accident Vehicle Emergency System

Dissertação apresentada ao Instituto Politécnico de Castelo Branco para cumprimento dos requisitos necessários à obtenção do grau de Mestre em Desenvolvimento de Software e Sistemas Interactivos, realizada sob a orientação científica do Doutor Fernando Reinaldo Silva Garcia Ribeiro, Professor Adjunto da Unidade Técnico-Científica de Informática da Escola Superior de Tecnologia do Instituto Politécnico de Castelo Branco.

How mobile is tech talent? A case study of IT professionals based on data from LinkedIn. CEPS Special Report No. 140, July 2016

This study aims to provide independent and in-depth insights into how IT professionals move from one region to another within Europe and beyond. This study was carried out by Mikkel Barslund, Research Fellow, and Matthias Busse, Researcher in the Economic Policy research unit at CEPS. The work was commissioned by the business networking website LinkedIn, whose data analysts kindly provided the data used in this study, aggregated by region and in relative and anonymised terms. The authors are solely responsible for the findings and opinions expressed in this study. CEPS is an independent policy research institute in Brussels, whose mission is to produce sound policy research leading to constructive solutions to the challenges facing Europe today. The views expressed in this study are solely those of the authors and should not be attributed to CEPS or to any other institution with which they are associated or to the business networking website LinkedIn.

Modelli e metodi per il dimensionamento di buffer floor-storage in magazzini scaffalati.

In questa tesi sono stati studiati i fattori che influenzano l'utilizzo del buffer in un magazzino manuale. Per poter realizzare le analisi è stato realizzato un programma C# che simula la gestione del magazzino. Per ottimizzare l'assegnamento dei pallet alle location si è inoltre costruito un modello AMPL. L'obiettivo del modello è minimizzare la differenza tra le classi ottime dei codici in ingresso e i posti pallet ai quali questi vengono assegnati. Il programma C# oltre all'interfaccia con il risolutore AMPL Gurobi, deve interfacciarsi anche con il data base Access nel quale sono stati organizzati i dati necessari per l'analisi. Questi dati sono reali e relativi agli ingressi e alle uscite di un magazzino contenente materiale farmaceutico di due settimane di lavoro. Per facilitare e velocizzare lo studio le informazioni considerate sono quelle di due soli corridoi e non dell'intero magazzino. I risultati hanno evidenziato quattro fattori principali che influenzano la differenza tra un l'allocazione dei pallet in ingresso ad un magazzino. Questi elementi sono: costo di utilizzo del buffer, numero di location libere ad inizio simulazione, momento d'esecuzione del picking a magazzino e tipologia di location vute ad inizio della simulazione. Per ognuno di questi si è cercato, per quanto possibile, di individuare le implicazioni che quanto studiato può avere nella progettazione di un sistema di stoccaggio reale sia dal punto di vista logistico sia dal punto di vista economico. Non sempre le osservazioni reali alle quali si è arrivati hanno un risvolto pratico immediato, ma senza dubbio possono essere utili nello studio preliminare per la realizzazione di un sistema di stoccaggio. All'interno delle conclusioni sono state inserite anche possibili studi e approfondimenti futuri che possono essere eseguiti partendo dal progetto di tesi qui presentato.