Les ambiances dans la conception architecturale : une « histoire » de représentations

Over the years architects have tried to make representations to reveal their architecture. Therefore, they have to take account of sensations and individual experience. In this paper, we suggest a historical approach to identify main events which had consequences on the history of architectural atmosphere representations. This text presents the beginning of an ongoing work about the overall representation of architectural atmosphere in which we wonder how architects create and communicate the atmosphere that they imagine in their future buildings. How do they deal with architectural atmosphere contingent at the different steps of the architectural project? How do they represent atmosphere they want to communicate? How do users feel the atmosphere in the building?L'architecte a cherché, au cours du temps, à donner une représentation évocatrice de sensations faisant référence à l'expérience personnelle pour révéler son architecture. Nous proposons dans cet article une approche historique qui permet d'identifier des leviers (évènements, écrits, outils, ...) qui ont fait évoluer la représentation des ambiances dans les différentes représentations architecturales. Il s'agit ici des prémisses d'un travail de recherche plus global en cours interrogeant la manière dont un architecte conçoit et communique ses intentions d'ambiances et la manière dont elles sont vécues dans le bâtiment. Comment les ambiances sont abordées dans les différentes phases du projet ? Comment sont-elles représentées dans le but d'être communiquées ? Comment sont-elles ressenties par les usagers une fois le bâtiment vécu

Qual a relação entre a cultura e o patrimônio? Uma parceria cultural no contexto museal com o meio escolar de aprendizagem de francês

Este artigo apresenta a análise de um programa educativo museal concebido e realizado pelo Centre d’histoire de Montréal e pelo Musée de la Personne, direcionado a jovens e adolescentes imigrantes, oriundos de diferentes comunidades culturais e matriculados em classes de aprendizagem de francês. O processo de avaliação se inscreve numa lógica de compartilhamento, estabelecendo-se uma colaboração entre universidade, instituições museais e meio escolar. O trabalho se desenvolve no laboratório de história e patrimônio de Montreal, com vistas à troca de experiências e conhecimentos por parte de atores sociais, tanto do mundo universitário quanto por parceiros culturais. Especificamente, a análise focou na relação entre cultura e patrimônio perceptível no programa, assim como nas modalidades de recepção e apropriação destes conteúdos pelos alunos das escolas participantes.

Qual a relação entre a cultura e o patrimônio? Uma parceria cultural no contexto museal com o meio escolar de aprendizagem de francês

Este artigo apresenta a análise de um programa educativo museal concebido e realizado pelo Centre d’histoire de Montréal e pelo Musée de la Personne, direcionado a jovens e adolescentes imigrantes, oriundos de diferentes comunidades culturais e matriculados em classes de aprendizagem de francês. O processo de avaliação se inscreve numa lógica de compartilhamento, estabelecendo-se uma colaboração entre universidade, instituições museais e meio escolar. O trabalho se desenvolve no laboratório de história e patrimônio de Montreal, com vistas à troca de experiências e conhecimentos por parte de atores sociais, tanto do mundo universitário quanto por parceiros culturais. Especificamente, a análise focou na relação entre cultura e patrimônio perceptível no programa, assim como nas modalidades de recepção e apropriação destes conteúdos pelos alunos das escolas participantes.

Yeast derivatives: a promising alternative for white wine oxidation prevention

In the previous decade, the use of Yeast Derivatives (YD) was proposed as a new strategy to control wine oxidation (Comuzzo et al., 2015). These products are obtained from yeasts by autolytic or hydrolytic processes and then dried to obtain the commercial products. The aim of this work was to carry out a preliminary investigation of commercial YDs with different compositions in order to (i) compare their capacity to prevent white wine oxidation in comparison with conventional treatment using SO2, and (ii) evaluate their impact on wine quality

High prevalence of PRPH2 in autosomal dominant retinitis pigmentosa in France and characterization of biochemical and clinical features.

International audiencePURPOSE:To assess the prevalence of PRPH2 in autosomal dominant retinitis pigmentosa (adRP), to report six novel mutations, to characterize the biochemical features of a recurrent novel mutation and to study the clinical features of adRP patients.DESIGN:Retrospective clinical and molecular genetic study.METHODS:Clinical investigations included visual field testing, fundus examination, high-resolution spectral-domain optical coherence tomography (OCT), fundus autofluorescence imaging and electroretinogram (ERG) recording. PRPH2 was screened by Sanger sequencing in a cohort of 310 French families with adRP. Peripherin-2 protein was produced in yeast and analyzed by Western blot.RESULTS:We identified 15 mutations, including 6 novel and 9 previously reported changes in 32 families, accounting for a prevalence of 10.3% in this adRP population. We showed that a new recurrent p.Leu254Gln mutation leads to protein aggregation, suggesting abnormal folding. The clinical severity of the disease in examined patients was moderate with 78% of the eyes having 1 to 0.5 of visual acuity and 52% of the eyes retaining more than 50% of the visual field. Some patients characteristically showed vitelliform deposits or macular involvement. In some families, pericentral RP or macular dystrophy were found in family members while widespread RP was present in other members of the same families.CONCLUSIONS:The mutations in PRPH2 account for 10.3% of adRP in the French population, which is higher than previously reported (0-8%) This makes PRPH2 the second most frequent adRP gene after RHO in our series. PRPH2 mutations cause highly variable phenotypes and moderate forms of adRP, including mild cases which could be underdiagnosed

Cost-effective sequence analysis of 113 genes in 1,192 probands with retinitis pigmentosa and Leber congenital amaurosis

Introduction: Retinitis pigmentosa (RP) and Leber congenital amaurosis (LCA) are two groups of inherited retinal diseases (IRDs) where the rod photoreceptors degenerate followed by the cone photoreceptors of the retina. A genetic diagnosis for IRDs is challenging since &gt;280 genes are associated with these conditions. While whole exome sequencing (WES) is commonly used by diagnostic facilities, the costs and required infrastructure prevent its global applicability. Previous studies have shown the cost-effectiveness of sequence analysis using single molecule Molecular Inversion Probes (smMIPs) in a cohort of patients diagnosed with Stargardt disease and other maculopathies. Methods: Here, we introduce a smMIPs panel that targets the exons and splice sites of all currently known genes associated with RP and LCA, the entire RPE65 gene, known causative deep-intronic variants leading to pseudo-exons, and part of the RP17 region associated with autosomal dominant RP, by using a total of 16,812 smMIPs. The RP-LCA smMIPs panel was used to screen 1,192 probands from an international cohort of predominantly RP and LCA cases. Results and discussion: After genetic analysis, a diagnostic yield of 56% was obtained which is on par with results from WES analysis. The effectiveness and the reduced costs compared to WES renders the RP-LCA smMIPs panel a competitive approach to provide IRD patients with a genetic diagnosis, especially in countries with restricted access to genetic testing.</p

Cost-effective sequence analysis of 113 genes in 1,192 probands with retinitis pigmentosa and Leber congenital amaurosis

Introduction: Retinitis pigmentosa (RP) and Leber congenital amaurosis (LCA) are two groups of inherited retinal diseases (IRDs) where the rod photoreceptors degenerate followed by the cone photoreceptors of the retina. A genetic diagnosis for IRDs is challenging since >280 genes are associated with these conditions. While whole exome sequencing (WES) is commonly used by diagnostic facilities, the costs and required infrastructure prevent its global applicability. Previous studies have shown the cost-effectiveness of sequence analysis using single molecule Molecular Inversion Probes (smMIPs) in a cohort of patients diagnosed with Stargardt disease and other maculopathies. Methods: Here, we introduce a smMIPs panel that targets the exons and splice sites of all currently known genes associated with RP and LCA, the entire RPE65 gene, known causative deep-intronic variants leading to pseudo-exons, and part of the RP17 region associated with autosomal dominant RP, by using a total of 16,812 smMIPs. The RP-LCA smMIPs panel was used to screen 1,192 probands from an international cohort of predominantly RP and LCA cases. Results and discussion: After genetic analysis, a diagnostic yield of 56% was obtained which is on par with results from WES analysis. The effectiveness and the reduced costs compared to WES renders the RP-LCA smMIPs panel a competitive approach to provide IRD patients with a genetic diagnosis, especially in countries with restricted access to genetic testing.This study received funding from Novartis. The funder was not involved in the study design, collection, analysis, interpretation of data, the writing of this article or the decision to submit it for publication. This work was supported by grants from Foundation Fighting Blindness Career Development Award CDGE-0621-0809-RAD (SR), Foundation Fighting Blindness project program award PPA-0123-0841-UCL (SR and SdB), Retinitis Pigmentosa Fighting Blindness, Fight for Sight UK (RP Genome Project GR586), Ghent University Special Research Fund (BOF20/GOA/023) (EDB and BL); EJP RD Solve-RET EJPRD19-234 (EDB, BL, SB, CR, FC, and SR). EDB (1802220N) and BL (1803816N) are FWO Senior Clinical Investigators of the Research Foundation Flanders (FWO). EDB, BL, SB, FC, and SR are members of ERN-EYE (Framework Partnership Agreement No. 739534)