The Dilemma of Basia's Pendant: European Community Design and the Protection of an Artist's Creations and Intellectual Property

This article is a description of the process of protecting a designer’s intellectual property through registration with the European Union Intellectual Property Office or EUIPO or through relying on the status of an Unregistered Community Design. The article discusses the registration process as well as the process of invalidation and considers issues relating to the enforcement of rights. Finally, the article focuses on whether a complaint about possible copyright infringement may be lodged against an online retailer or an online e-commerce website that offers unlicensed sales or unauthorized copies of a designer’s design by utilizing the descriptions or photographs of the designer

On the peak strength of 7050 aluminum alloy: mechanical and corrosion resistance

This work consists of an experimental study on the ageing response and resulting properties of AA7050 plate material. New heat treatments are investigated for achieving a peak-aged temper, as a T6 temper may be said to be, that achieves yield and tensile strengths superior to those of the documented T7 treatments. For this alloy, the Standard establishes T7X tempers which were developed to obtain a very good compromise between mechanical strength and corrosion resistance. Nevertheless, for all those applications in which the environment is not considered critical for corrosion behaviour, the peak strength condition could be beneficial. In this experimental work, the authors use standard hardness testing to investigate mechanical response as a function of ageing time at several ageing temperatures, all applied immediately after solution. Upon identifying specific times and temperatures of interest, specimens aged under the selected treatments were subjected to tensile testing and intergranular corrosion testing. The results show that a single-step ageing heat treatment is able to produce a significantly high both yield and ultimate tensile strength. Moreover, the corrosion test data indicates that this new heat treatment produces corrosion resistance similar to that of the T76 heat treatment

Novel system for distributed energy generation from a small scale concentrated solar power

The present work describes the realization of a modular 1-3 kWe, 3-9 kWth micro Combined Heat and Power (m-CHP) system based on innovative Concentrated Solar Power (CSP) and Stirling engine technology. The cogeneration of energy at distributed level is one of leading argument in large part of energy policies related to renewable energy resources and systems. This CSP m-CHP will provide electrical power, heating and cooling for single and multiple domestic dwellings and other small buildings. The developed system integrates small-scale concentrator optics with moving and tracking components, solar absorbers in the form of evacuated tube collectors, a heat transfer fluid, a Stirling engine with generator, and heating and/or cooling systems; it incorporates them into buildings in an architecturally acceptable manner, with low visual impact. Some good results have already been achieved, while developments on several technology subcomponents will be finalized through first part of 2013. Two Cer.Met. have been modelled, realized and tested. The up scaled receiver, in form of Cer.Met. coating based on TiO2 - Nb, has been confirmed an absorptance of 0.94 and emittance of 0.1 (@350°C). A second Cer.Met. coating based on SiO2 - W has demonstrated an absorptance of 0.93 and emittance of 0.09 (@350°C). A full-evacuated solar tube has been designed and realized, with absorber of 12 mm in diameter and length in 2 meters. The system is provided of a concentration ratio 12:1, and a single module is 200 cm long, 40 cm wide and 20-25 cm high. Two or more modules can be combined. The evacuated solar tube, located on the focus, has the selective absorber on a tube of 12 mm in diameter. A very thin glass mirror has been developed (< 1 mm). The overall mirror reflectivity has been measured, the verified value is 0,954. Research has proposed a high energy density, double acting Stirling engine, provided of innovative heat exchangers realized through Selective Laser Melting process. The engine is a low speed (250 RPM), high pressure (130 Bars) and compact solution able to be run at 300°C and generate 3,5 kW nominal power. The solar technology has actually entered the proof-of-concept stage. A solar plant has been installed in Malta, by Arrow Pharm company, supplying the industrial process of generated steam at 180°C and 3.5 absolute pressure. The solar collector's efficiency is close to 47% in presence of 900 W/m2 of direct solar radiation. During 2013, solar evacuated tubes with innovative Cer.Met. coating, together with new thin glass mirrors will upgrade the demonstration site, together with a new and innovative low temperature difference and high energy density Stirling. By end-2013, the system will be demonstrated, with the overall objective to achieve a minimum of 65% in solar collectors' efficiency at 300°C, and 12 - 15% of overall electrical efficiency by the Stirling cycle.peer-reviewe

Une arme miracle contre le chômage ?:Une nouvelle évaluation des effets des allègements de charges sociales sur les bas salaires

La revue Économie et Statistique vient de faire paraître un article de Bruno Crépon et Rozenn Desplatz qui propose une nouvelle évaluation des effets des allègements de charges sociales sur les bas salaires. Selon cette étude, ceux-ci auraient provoqué, de 1994 à 1997, une augmentation de 460 000 du nombre d’emplois dans l’économie française. Ce chiffre est nettement supérieur aux estimations antérieures 1. Il est maintenant évoqué dans le débat public pour justifier la poursuite de la politique de baisse des charges sociales, comme axe central de la politique de l’emploi. Compte tenu de la complexité de l’article, la plupart des commentateurs se contentent de citer le chiffre tel quel : « Les experts ont parlé ». Mais quelle est la fiabilité de ce résultat ? Par quels mécanismes, ces emplois ont-ils été créés ? La réduction des cotisations sociales est-elle une arme miracle contre le chômage ? [Premier paragraphe]Bruno Crépon et Rozenn Deslpatz, Une nouvelle évaluation des effets des allègements de charges sociales sur les bas salaires, Economie et Statistique, n°348, 2001, 0336-145

Distributed m-CHP generation from a small scale concentrated solar power installation

The present work describes the realization of a modular 1-3 kWe, 3-9 kWth micro Combined Heat and Power (m-CHP) system based on innovative Concentrated Solar Power (CSP) and Stirling engine technology. The cogeneration of energy at distributed level is one of leading argument in large part of energy policies related to renewable energy resources and systems. This CSP m-CHP will provide electrical power, heating and cooling for single and multiple domestic dwellings and other small buildings. The developed system integrates small-scale concentrator optics with moving and tracking components, solar absorbers in the form of evacuated tube collectors, a heat transfer fluid, a Stirling engine with generator, and heating and/or cooling systems; it incorporates them into buildings in an architecturally acceptable manner, with low visual impact. Some good results have already been achieved, while developments on several technology subcomponents will be finalized through first part of 2013. Two Cer.Met. have been modelled, realized and tested. The up scaled receiver, in form of Cer.Met. coating based on TiO2 - Nb, has been confirmed an absorptance of 0.94 and emittance of 0.1 (@350C). A second Cer.Met. coating based on SiO2 - W has demonstrated an absorptance of 0.93 and emittance of 0.09 (@350C). A full-evacuated solar tube has been designed and realized, with absorber of 12 mm in diameter and length in 2 meters. The system is provided of a concentration ratio 12:1, and a single module is 200 cm long, 40 cm wide and 20-25 cm high. Two or more modules can be combined. The evacuated solar tube, located on the focus, has the selective absorber on a tube of 12 mm in diameter. A very thin glass mirror has been developed (< 1 mm). The overall mirror reflectivity has been measured, the verified value is 0,954. Research has proposed a high energy density, double acting Stirling engine, provided of innovative heat exchangers realized through Selective Laser Melting process. The engine is a low speed (250 RPM), high pressure (130 Bars) and compact solution able to be run at 300C and generate 3,5 kW nominal power. The solar technology has been fully proven in a demonstration site. The solar plant has been installed in Malta, by Arrow Pharm company, supplying the industrial process of generated steam at 180C and 3.5 absolute pressure in a first phase and supporting the tests of improved technologies at a second stage. The solar collector's efficiency is around 50% in presence of 900 W/m2 of direct solar radiation and at 300C. During 2013, solar evacuated tubes with innovative Cer.Met. coating, together with new thin glass mirrors has upgraded the demonstration site, together with a new and innovative low temperature difference and high energy density Stirling. By end-2013, the system has been demonstrated. At the beginning of 2014 it will be transferred to Trento for further optimization, with the overall objective to achieve a minimum of 65% in solar collectors efficiency at 300C, and 12-15% of overall electrical efficiency by the Stirling cycle. The actual work is part of a FP7 European Funded project, DIGESPOBajada New Energy, General Membrane, EcoGroup, Econetique, Energy Investment, JMV Vibro Blocks, Solar Engineering, Solar Solutions.peer-reviewe

Functional Characterization of Two Variants at the Intron 6-Exon 7 Boundary of the KCNQ2 Potassium Channel Gene Causing Distinct Epileptic Phenotypes

Pathogenic variants in KCNQ2 encoding for Kv7.2 potassium channel subunits have been found in patients affected by widely diverging epileptic phenotypes, ranging from Self-Limiting Familial Neonatal Epilepsy (SLFNE) to severe Developmental and Epileptic Encephalopathy (DEE). Thus, understanding the pathogenic molecular mechanisms of KCNQ2 variants and their correlation with clinical phenotypes has a relevant impact on the clinical management of these patients. In the present study, the genetic, biochemical, and functional effects prompted by two variants, each found in a non-familial SLNE or a DEE patient but both affecting nucleotides at the KCNQ2 intron 6-exon 7 boundary, have been investigated to test whether and how they affected the splicing process and to clarify whether such mechanism might play a pathogenetic role in these patients. Analysis of KCNQ2 mRNA splicing in patient-derived lymphoblasts revealed that the SLNE-causing intronic variant (c.928-1G > C) impeded the use of the natural splice site, but lead to a 10-aa Kv7.2 in frame deletion (Kv7.2 p.G310Δ10); by contrast, the DEE-causing exonic variant (c.928G > A) only had subtle effects on the splicing process at this site, thus leading to the synthesis of a full-length subunit carrying the G310S missense variant (Kv7.2 p.G310S). Patch-clamp recordings in transiently-transfected CHO cells and primary neurons revealed that both variants fully impeded Kv7.2 channel function, and exerted strong dominant-negative effects when co-expressed with Kv7.2 and/or Kv7.3 subunits. Notably, Kv7.2 p.G310S, but not Kv7.2 p.G310Δ10, currents were recovered upon overexpression of the PIP2-synthesizing enzyme PIP5K, and/or CaM; moreover, currents from heteromeric Kv7.2/Kv7.3 channels incorporating either Kv7.2 mutant subunits were differentially regulated by changes in PIP2 availability, with Kv7.2/Kv7.2 G310S/Kv7.3 currents showing a greater sensitivity to PIP2 depletion when compared to those from Kv7.2/Kv7.2 G310Δ10/Kv7.3 channels. Altogether, these results suggest that the two variants investigated differentially affected the splicing process at the intron 6-exon 7 boundary, and led to the synthesis of Kv7.2 subunits showing a differential sensitivity to PIP2 and CaM regulation; more studies are needed to clarify how such different functional properties contribute to the widely-divergent clinical phenotypes

Development of W–SiO2 and Nb–TiO2 solar absorber coatings for combined heat and power systems at intermediate operation temperatures

Two new absorber coatings for mid-temperature operation (300–350 1C) in collectors for solar thermal electricity plants are presented in this study. The absorbers consist of two cermet layers of either W–SiO2 or Nb–TiO2, deposited on a molybdenum infrared reflector and coated with an antireflection layer of silicon oxide. The optimization of the optical performance was made in two steps. First, the layer structure was optimized in model calculations. The optical constants used in this modelling were derived directly from sputtered films of the cermet constituents using reflectance and transmittance measure- ments. The absorber coatings were then sputter-deposited using parameters from the modelling. The results show good agreement between modelled and sputtered optical performance evaluated as solar absorptance and thermal emittance at 350 1C. The optimal values reached for W–SiO2 was 0.91 in combination with 0.08 and 0.93 in combination with 0.09 for Nb–TiO2. The materials characterization from XRD, AES and TEM shows that the composite coatings contain nano-metal inclusions, meaning that they are cermet coatings. Scratch tests show that the coatings adhere well to the substrate of stainless steel. Temperature testing at 350 1C in vacuum for up to 1500 h shows that both coatings are stable under such conditions. Only a slight change occurs during the first 72 h that decreases the emittance but does not change the solar absorptance. ERDA confirms that there is no detectable level of ion migration between layers, only a small decrease in hydrogen content was observed, which indicates outgassing.Digespo projec

Brain-derived tau: a novel blood-based biomarker for Alzheimer's disease-type neurodegeneration

Blood-based biomarkers for amyloid beta and phosphorylated tau show good diagnostic accuracies and agreements with their corresponding CSF and neuroimaging biomarkers in the amyloid/tau/neurodegeneration [A/T/(N)] framework for Alzheimer's disease. However, the blood-based neurodegeneration marker neurofilament light is not specific to Alzheimer's disease while total-tau shows lack of correlation with CSF total-tau. Recent studies suggest that blood total-tau originates principally from peripheral, non-brain sources. We sought to address this challenge by generating an anti-tau antibody that selectively binds brain-derived tau and avoids the peripherally expressed 'big tau' isoform. We applied this antibody to develop an ultrasensitive blood-based assay for brain-derived tau, and validated it in five independent cohorts (n = 609) including a blood-to-autopsy cohort, CSF biomarker-classified cohorts and memory clinic cohorts. In paired samples, serum and CSF brain-derived tau were significantly correlated (rho = 0.85, P < 0.0001), while serum and CSF total-tau were not (rho = 0.23, P = 0.3364). Blood-based brain-derived tau showed equivalent diagnostic performance as CSF total-tau and CSF brain-derived tau to separate biomarker-positive Alzheimer's disease participants from biomarker-negative controls. Furthermore, plasma brain-derived tau accurately distinguished autopsy-confirmed Alzheimer's disease from other neurodegenerative diseases (area under the curve = 86.4%) while neurofilament light did not (area under the curve = 54.3%). These performances were independent of the presence of concomitant pathologies. Plasma brain-derived tau (rho = 0.52-0.67, P = 0.003), but not neurofilament light (rho = -0.14-0.17, P = 0.501), was associated with global and regional amyloid plaque and neurofibrillary tangle counts. These results were further verified in two memory clinic cohorts where serum brain-derived tau differentiated Alzheimer's disease from a range of other neurodegenerative disorders, including frontotemporal lobar degeneration and atypical parkinsonian disorders (area under the curve up to 99.6%). Notably, plasma/serum brain-derived tau correlated with neurofilament light only in Alzheimer's disease but not in the other neurodegenerative diseases. Across cohorts, plasma/serum brain-derived tau was associated with CSF and plasma AT(N) biomarkers and cognitive function. Brain-derived tau is a new blood-based biomarker that outperforms plasma total-tau and, unlike neurofilament light, shows specificity to Alzheimer's disease-type neurodegeneration. Thus, brain-derived tau demonstrates potential to complete the AT(N) scheme in blood, and will be useful to evaluate Alzheimer's disease-dependent neurodegenerative processes for clinical and research purposes

Gabapentin treatment in a patient with KCNQ2 developmental epileptic encephalopathy

De novo variants in KCNQ2 encoding for Kv7.2 voltage-dependent neuronal potassium (K+) channel subunits are associated with developmental epileptic encephalopathy (DEE). We herein describe a the clinical and electroencephalographic (EEG) features of a child with early-onset DEE caused by the novel KCNQ2 p.G310S variant. In vitro experiments demonstrated that the mutation induces loss-of-function effects on the currents produced by channels incorporating mutant subunits; these effects were counteracted by the selective Kv7 opener retigabine and by gabapentin, a recently described Kv7 activator. Given these data, the patient started treatment with gabapentin, showing a rapid and sustained clinical and EEG improvement over the following months. Overall, these results suggest that gabapentin can be regarded as a precision therapy for DEEs due to KCNQ2 loss-of-function mutations