Les lysosomes sont importants pour la survie et la fonction des cellules du système nerveux central et en particulier des neurones. Le mécanisme de la reformation des lysosomes est crucial pour maintenir une quantité adéquate de lysosomes fonctionnels dans les cellules. La spatacsine, qui joue un rôle dans le ce mécanisme est impliquée dans la paraplégie spastique de type SPG11 ; une maladie caractérisée par des troubles moteurs et cognitifs sévères. L’utilisation de modèles cellulaires de cette pathologie permet d’étudier les mécanismes physiopathologiques à l’origine d’altérations de la reformation des lysosomes. J’ai montré que la perte de fonction de la spatacsine est responsable de l’accumulation de lipides dans les lysosomes. Ces accumulations sont constituées de gangliosides et de cholestérol et sont présentes dans les autolysosomes perturbant leur recyclage en lysosomes, notamment en empêchant le recrutement de protéines impliquées dans le mécanisme. Les accumulations de gangliosides rendent les neurones à l’exposition au glutamate ce qui suggère que ces altérations pourraient avoir un rôle dans la neurodégénérescence. J’ai aussi montré que l’absence de spatacsine provoque une dérégulation de l’import de Ca2+ extracellulaire par le « store-operated calcium entry » ce qui conduit à altération de l’homéostasie calcique. L’inhibition de l’import de calcium par le SOCE permet de réduire les accumulations de lipides et de rétablir partiellement le recyclage des lysosomes. Ainsi, l’absence de spatacsine induit une altération de l’homéostasie calcique qui participe à l’accumulation de lipides dans le système lysosomal ce qui est délétère pour la survie des neurones. / Lysosomal dysfunctions are involved in a large number of neurodegenerative diseases highlightingthe crucial function of lysosomes in neuron survival and function. The mechanism of lysosomereformation from autolysosomes allow cells to maintain the ool of functional lysosomes.Disruptions of this rocess are involved in athologies affecting the central nervous system. Inparticular, spatacsin that lays a role in lysosome recycling is implicated in hereditary spasticparaplegia type SPG11, a severe disease characterized by motors and cognitive alterations. Thispathology is caused by loss of function mutations in SPG11, encoding spatacsin. The study ofSPG11 cellular models gives the opportunity to decipher the hysiopathological mechanismsunderlying lysosomal reformation disruptions.During my thesis, I showed that loss of spatacsin function induces lipid accumulation in lysosomesand articularly in autolysosomes both in fibroblasts and neurons from Spg11-/- mice. Gangliosidesand cholesterol are among lipids that accumulate in autolysosomes impairing lysosomal membranerecycling by disrupting the recruitment of keys roteins. Neurons with ganglioside accumulationsare more sensitive to glutamate induced neuronal death, suggesting that these accumulations areinvolved in neurodegeneration. These results could be of great importance since accumulations ofgangliosides in lysosomes arise in many diseases.I also showed that loss of spatacsin disrupts extracellular calcium import by the store-operatedcalcium entry (SOCE) leading to an increase in cytosolic calcium levels. Lysosomal calcium contentis also increased in Spg11-/- cells and calcium release from lysosome by TRPML1 is reduced.Inhibiting SOCE and stimulating lysosomal calcium release by TRPML1 reduced lipidsaccumulations in lysosomes and artially restored lysosome reformation.Our data suggest that absence of spatacsin is responsible for a disruption of calcium homeostasisthat contributes to lipid accumulation in autolysosomes, disturbing reformation of lysosomes fromautolysosomes. Inhibiting gangliosides synthesis could be used as a therapeutic strategy. However,understanding how loss of function of spatacsin alters these cellular athways will allow thedevelopment of targeted therapeutic approaches.

Les distributeurs se caractérisent désormais comme de véritables marques qui cherchent à construire leur capital pour délivrer davantage de valeur aux consommateurs et ainsi tenter de les satisfaire, de les fidéliser et finalement d'améliorer leur performance. Si de nombreuses recherches académiques et managériales insistent sur la pertinence pour les entreprises de développer leur capital-marque, d'autres investigations rappellent la complexité dune telle tâche et les nombreuses difficultés afférentes à la mesure. Le distributeur n'échappe pas à cette complexité, pis encore, les spécificités de la distribution et les comportements des consommateurs dans ce secteur requièrent davantage de précaution dans l'application du concept aux firmes de distribution. L'objectif de ce travail porte donc sur la définition, la conceptualisation et la mesure du capital-marque du distributeur. Pour ce faire, une analyse qualitative exploratoire est tout d'abord menée afin de compléter la revue de littérature, d'explorer les perceptions et comportements des consommateurs et de faire émerger les variables d'action du distributeur grâce auxquelles il peut générer de la valeur. Afin de pallier aux insuffisances théoriques et de proposer un outil opérationnel aux managers, un cadre conceptuel du capital-marque adapté au distributeur est ensuite construit sur la complémentarité des apports de la littérature et de l'étude exploratoire. Puis le test empirique de ce modèle, par l'usage d'un questionnaire administré auprès de 313 consommateurs et d'une analyse avec l'approche PLS, permet de confirmer le cadre conceptuel défini. Enfin, la discussion des résultats suggèrent que les distributeurs peuvent construire leur marque sur huit dimensions distinctes qu'elles soient relatives aux produits vendus, aux points de vente ou aux expériences de magasinage des consommateurs. Plus spécifiquement, l'apparence du magasin et la qualité des produits contribuent le plus fortement à la création de valeur / Nowadays, retailers must be considered as real and unique brands that wish to build their equity. By enhancing the value offered to consumers, they can retain and attract them and consequently, increase their own performance. However, the brand equity measure is complex and many specificities arise from the retail area that clearly call for a specific conceptualization. The aim of this research is to define, conceptualize and measure the equity of the retailer as a brand. In that respect, an exploratory qualitative research is conducted to firstly, obtain a better understanding of consumers' perceptions and behaviors and secondly, to identify the variables the retailer can leverage to build its equity. Then, a new conceptual framework is established. It includes eight variables related to the product assortment, the outlet or the consumer experience. The model is estimated by using a survey based upon 313 consumer responses and the PLS path modeling approach. The results provide evidence that retailers can build their equity on these eight dimensions. More specifically, the outlet interior appearance and the product quality are the two main drivers of retailer brand equity

Online stores today need to be attractive to compete on the rapid growing market of online businesses. Standardized online store software solutions sold from IT companies to small businesses are also growing making it easier for anyone creating an online store. Less attention has been paid to how well these software solutions help the administrators to create a competitive online store regarding the administrator’s limited knowledge about usability and web design. This research intends to develop knowledge about how well two online store solutions perform from evaluation criteria within online shopping, usability and web design and how an online store solution for small businesses can benefit from a comparison with another online store solution sold to a larger range of businesses. The two products are evaluated and compared through Heuristic evaluations and A/B testing with 5 usability experience (UX) experts and analyzed with Trend analysis. The results show strengths in the solutions where they both were easy to learn, had easy access to help and good tools for creating loyal customers and weaknesses in inspiring, navigation and lack in mobile adaption. The findings suggest how the online store solution for small businesses can improve their statistics and design colors from the comparison of the larger product. A limitation in the study is that all evaluators come from the same team. For future work more cultural studies can be conducted for online stores in western countries, and user tests with administrators of online shops.

The aim of this bachelor thesis is to examine which experiential dimensions that are perceived as important for customers and how these dimensions are delivered by Swedish beauty and makeup retailers. The focus is to examine how their physical in-store environments succeeds with delivering extraordinary customer experiences. The thesis is conducted through a deductive approach and a quantitative research method have been implemented. Further on, the software Statistical Package for Social Science (SPSS) have been used to process the data collected from an online questionnaire. The empirical findings were then calculated into six variables that were analyses and discussed in relation to existing theories to strengthen and improve the research of customer experience. The thesis found that experiential dimensions of interaction, emotion, differentiation and in-store technologies are considered as important by customers within the age of 18-30, when creating extraordinary customer experiences in the context of beauty and makeup in-store environments. Moreover, the collected data prove that Swedish Beauty and makeup stores fails to deliver these four dimensions in the extent to create extraordinary customer experiences. Customers of today want new and interesting in-store environments, which means that retailers need to permanently elaborate their environments with unique design element within these dimensions to deliver memorable customer experiences. The result from this thesis can be used by beauty and makeup retailers and other industries to increase their delivery of extraordinary customer experience.

Die Verfügbarkeit neuer Technologien wie Multi-Core, SSD oder große Hauptspeicherkapazitäten bieten eine Gelegenheit, die klassischen Architekturansätze von Datenbanksystemen zu überdenken und an bestimmten Stellen zu korrigieren. In diesem Beitrag stellen wir die Grobstruktur der neuen hauptspeicherzentrierten SAP Technologie als einen Ansatz einer kommerziellen Umsetzung moderner Architekturkonzepte vor. Zentrales Design-Kriterium ist dabei ein hybrider Ansatz, um eine möglichst hohe Anzahl von Anforderungsvarianten optimal zu unterstützen. Nach einer Einleitung führt der Artikel durch die wichtigsten Architekturkomponenten und illustriert den grundsätzlichen Aufbau des Systems. Für einen „deep dive“ werden zwei Bereiche in Teil 3 und 4 des Artikels im Detail diskutiert. Dabei greift der Artikel zum einen den Aspekt der physischen Optimierung im Kontext eines hauptspeicherzentrierten Systems auf und diskutiert unterschiedliche Komprimierungs- und Sortierungskriterien, wie sie im klassischen disk-zentrierten Ansatz nicht zu finden sind. Zum anderen wird die Unterstützung von Planungsanwendungen skizziert, wodurch ein Einblick in die spezifische Unterstützung einer Anwendungsdomäne („business planning“) und die prinzipiellen Erweiterungen für komplexe Operationen zur direkten Unterstützung von darauf aufbauender Planungsfunktionalität gezeigt werden.

Grocery stores are an important amenity in neighborhoods and access to grocery stores is important for health and well-being. While grocery store accessibility is a popular topic of research, studies measuring access for pedestrians, bicyclists, and transit riders are extremely rare. When a new store opened in the Excelsior/Outer Mission districts of San Francisco on a street lacking basic infrastructure for pedestrians, bicyclists, and transit riders, the importance of this study became apparent. The Excelsior/Outer Mission neighborhood has a shocking number of collisions (over 1,100 between 2015 and 2019), elevated levels of walking, biking, and transit ridership, and minimal safe infrastructure for these modes compared to other residential areas in San Francisco. To account for the effects of these conditions on accessibility, a rating system to measure infrastructure for users was used in addition to the more traditional gravity model. Combining results into a composite accessibility score highlights how using only a gravity model to measure accessibility may conceal some of the nuances of accessibility as perceived by pedestrians, bicyclists, and transit riders. While it appears from gravity indices that stores in the Excelsior/Outer Mission are only slightly less accessible, the sensitivity analysis shows that infrastructure can have a large effect on overall accessibility. Specifically in the Excelsior/Outer Mission neighborhood, with its rather low infrastructure scores, the higher the weight attributed to the importance of infrastructure the lower is composite accessibility. Rather than measuring accessibility using only travel time via a gravity model or other spatial model, this study shows the importance of combining physical proximity measurements with infrastructure information to provide a more complete picture. This is particularly important for those walking, biking, or riding transit where safety is an important consideration. This study provides one such way to include the unique considerations of pedestrians, bicyclists, and transit riders by including an infrastructure scoring system. Not only does this highlight the importance of including infrastructure measures, but it provides a framework for future infrastructure improvements around stores.

L’une des voies d’innovation plébiscitées par les détaillants pour orchestrer l’évolution de leur proposition de valeur consiste à réviser le design des espaces de vente. Les contributions académiques sur l’expérience en magasin se sont concentrées sur les perceptions des consommateurs ainsi que sur le repérage de pratiques managériales significatives. En prenant pour objet la traduction du repositionnement d’une enseigne par le design expérientiel des espaces de vente, la thèse propose de suivre le processus au cœur même du marché et de mettre à jour les mécanismes qui le sous-tendent. La conduite d’une étude longitudinale de cas enchâssés dévoile une conception holistique, basée sur l’interaction souhaitée du chaland avec le magasin. L’analyse relate l’enchâssement des quatre phases du cycle de vie et permet de recenser pour chaque phase les événements et problématiques associés aux six dimensions du processus. La thèse établit le rôle prégnant de la vision entrepreneuriale du dirigeant, clé de voûte de l’innovation. La proposition de valeur se matérialise par trois composantes gigognes que sont l’intrigue, l’action et le décor. Pour chacune des cinq étapes du parcours-client, des éléments de décor sont implantés pour relayer ou renforcer l’action souhaitée. Ces éléments constituent des mécanismes ou dispositifs destinés à stimuler le système expérientiel du chaland. L’évaluation, qui porte sur la mesure du positionnement perçu et vécu, contribue à ajuster la proposition de valeur au regard de quatre niveaux de cohérence et de la flexibilité du design. Enfin, les logiques de coproduction occasionnent une possible co-destruction de valeur, intentionnelle ou accidentelle. / One of the innovative ways favoured by retailers to drive change in their value proposition is to review the design of their stores. Academic contributions to the in-store experience have mostly focused on consumer perspective and identifying relevant managerial practices. The core of this research studies how repositioning a retail brand translates into the experiential design of retail spaces. To this end, the research follows the repositioning process from a managerial perspective and updates the mechanisms that underlie it. The longitudinal study of embedded cases reveals the importance of an holistic design that takes into account the desired interactions between the shopper and the store. The analysis shows the four overlapping phases of the store’s life cycle, and breaks down the process into six dimensions, each with its own events and issues. The six dimensions are vision, plotline, action, decor, assessment and coproduction.The entrepreneurial vision of the leader is the cornerstone of the whole innovation process. The value proposition is embodied by three components, namely plotline, action and decor. For each of the five stages of the shopper’s journey, elements of the decor are implemented to relay or reinforce the desired action. These are mechanisms or devices meant to stimulate the shopper’s experiential system. The assessment, which involves measuring the perceived and experienced positioning, helps to adjust the value proposition in terms of four levels of consistency and flexibility of design. Finally, the coproduction of store design between different stakeholders can cause a co-destruction of value, whether intentional or accidental.

Thesis (PhD (Consumer Science. Clothing and Textiles))--University of Stellenbosch, 2008. / The current apparel retail environment is marked by intense competitive activity. The key to survival is the implementation of effective differentiation strategies. Corporate and retail branding provides retailers with a powerful tool to differentiate themselves in the marketplace and store image is a vital component of this branding strategy. This exploratory study set out to investigate the underlying theoretical structure of store image. A store image scale was developed for the measurement of the perceived importance of store image. The study adopted a five phase methodology, namely (1) construct definition and domain specification, (2) generation and judging of measurement items, (3) purification of the store image scale, (4) assessing the reliability and validity of the store image scale, and (5) assessing the perceived importance of the dimensions of store image in selected discount and specialty stores through practical implementation of the store image scale. The first two phases of the study resulted in a Model of Store Image delineating the underlying structure of store image which formed the basis for a store image definition, as well as a 232-item store image scale with established content and face validity. Phase 3 comprised two pilot studies that served to purify the store image scale. The first pilot study concluded in a 214-item scale that was deemed too lengthy for practical implementation in the apparel retail environment. The second pilot study resulted in a 55-item store image scale that was deemed acceptable for practical implementation. Correlation analysis provided support for the shortened version of the store image scale. The scale was not representative of all the sub dimensions associated with store image. This was reflected in the Revised Model of Store Image. Phase 4 employed a mall-intercept research method. The sample population (n=534) consisted of apparel consumers, both male and female, between the ages of 20 and 60. They belonged to the black, coloured or white population groups who patronised specific apparel retail outlets. Trained fieldworkers gathered the data at selected discount and specialty apparel stores. Confirmatory factor analysis was performed on the data and results provided support for the reliability of the store image scale. The Atmosphere, Convenience, Institutional, and Promotion dimensions exhibited good model fit. The Facilities and Sales personnel dimensions, as well as the Revised Model of Store Image showed evidence of acceptable model fit. The Merchandise and Service dimensions demonstrated poor fit. Only the Sales personnel dimension showed convergent validity. Support was found for marginal convergent validity of the Atmosphere, Convenience, Facilities, Promotion, and Service dimensions, as well as the Revised Model of Store Image. The Institutional and Merchandise dimensions did not exhibit convergent validity. Apart from the Convenience and Service dimensions, discriminant validity for all dimensions was established. Item analysis identified seven scale items for deletion which could potentially result in better model fit of the individual dimensions as well as the Revised Model of Store Image. The deletion of these items could contribute to increased convergent and discriminant validity. For purposes of Phase 5 the data gathered during Phase 4 was submitted to statistical analysis. Results indicated that discount and specialty apparel store consumers ranked the Atmosphere, Promotion, Merchandise, Institutional, and Sales personnel dimensions similarly in perceived importance. Discount apparel store consumers ranked the Facilities and Convenience dimensions higher, whilst specialty consumers ranked the Service dimension higher. However, the differences in ranking for all dimensions remained relatively small for both store types. Statistical differences in the perceived importance of only two dimensions, namely the Institutional and Service dimensions were found. The study culminated in revised 48-item store image scale. A Final Model of Store Image and definition of store image were proposed as point of departure for future research.. The main implications for retailers were formulated as: 􀂍 The Final Model of Store Image identified the dimensions and sub dimensions of store image. Retailers should manipulate the tangible and intangible store attributes associated with these dimensions and sub dimensions to build a favourable store image. Due to the gestalt nature of store image it is imperative that all store image dimensions are presented in a cohesive and consistent manner. 􀂍 The store image scale will enable retailers to ascertain which dimensions are salient to their target consumers. These dimensions should be incorporated in the retail strategy.

by Chung Shiu-ling, Shirley. / Bibliography: leaf 45 / Thesis (M.B.A.)--Chinese University of Hong Kong, 1986

by Wong Hang-Tat. / Thesis (M.B.A.)--Chinese University of Hong Kong, 1992. / ABSTRACT --- p.ii / TABLE OF CONTENTS --- p.iii / Chapter I --- INTRODUCTION --- p.1 / Petroleum Business in Hong Kong --- p.1 / Retail Market --- p.2 / Shell --- p.12 / Convenience Store Concept --- p.14 / Study Objectives --- p.18 / Chapter II --- TARGET GROUP --- p.23 / Chapter III --- METHODOLOGY --- p.26 / Chapter IV --- RESULTS & ANALYSIS --- p.33 / Customer perception --- p.33 / Investment --- p.41 / Profitability --- p.43 / Operations --- p.47 / Competitor Marketing Strategies --- p.49 / Chapter V --- RECOMMENDATIONS --- p.51 / Limitations --- p.51 / Recommendations --- p.52 / Conclusion --- p.58 / Further Work --- p.59 / APPENDIX --- p.60