Gotowa bibliografia na temat „Agences inbound”

This study aims to examine the effectiveness of international exhibition of tourism and travels “ SITEV” in improving the Algerian tourism destination image, and its impact on attractiveness of tourists, through the questionnaire that was sent by e-mail and Facebook, and it was conducted on a sample of responsible of Algerian travels and tourism agencies especially in inbound tourism and its participation in the 19th edition that was organized in 2018.The findings indicate that the SITEV has many positive aspects and reflects the history, authenticity and cultural heritage of Algeria tourism destination that it was contributing in improving Algeria tourism destination image. However, the weakness of media coverage and the high costs of venue are contributed to reluctance of foreign operators and tourists to participate in the last editions which will require great efforts and promotion campaigns across big media channels and diminishing costs.
 Cette étude vise à examiner l'efficacité du Salon International du Tourisme et des Voyages « SITEV » dans l'amélioration de l'image de la destination touristique Algérienne, et son impact sur l'attractivité des touristes étrangers. Et ce à travers le questionnaire qui a été envoyé par e-mail et Facebook, et qui a été réalisé chez un échantillon de responsables Algériens au niveau d'agences de voyages et de tourisme ; notamment les agences qui s’occupent des touristes étrangers et spécialement celles qui ont participés à la 19ème édition du Salon organisée en 2018.Les résultats obtenus indiquent que le SITEV dispose de nombreux aspects positifs et reflète réellement l'histoire, l'authenticité et le patrimoine culturel de l'Algérie. Ces éléments ont contribué à leur tour à l’amélioration de l'image de la destination touristique Algérienne. Néanmoins ; la faiblesse de la couverture médiatique et les coûts élevés de loyer des stands contribuent à la réticence des opérateurs étrangers et des touristes à participer surtout aux dernières éditions, ce qui nécessitera de grands efforts et des campagnes de promotion sur les grandes chaines médiatiques internationales spécialisées, ainsi que la révision des coûts de loyer des stands afin de les diminuer.

This study presented an overview of Bangladesh's tourism sector, which also strived to identify the opportunities and challenges that warrant policy interventions. Both primary and secondary data were used in this research. It was found that the frequency of inbound tourists' arrival showed a declining trend. The tourism sector's growth cannot keep pace with national growth domestic product though Bangladesh's global rank and score showed an increasing trend. Despite economic upsurging and vast potentiality, Bangladesh could not be a popular destination for foreign tourists. Cultural and natural resources and tourist and airport infrastructure have been identified as the disadvantaged pillars. The country cannot offer cheaper and more comfortable accommodation and transportation facilities. There is a lack of diversity in tourism events and fun-making elements. Online tourist platforms, booking, and financial transactions are not widely available and accessible. Supermall and safe food providing agencies have not been developed across the country. The study advocates for formulating a holistic national tourism policy eyeing achieving sustainable development goals. There is ample scope for developing a regional tourism hub taking Bhutan and Nepal.

Since the COVID-19 spread, tourism has been hit particularly hard globally, also in Albania, in particular inbound tourism with a drastic drop in tourist arrivals out from the country because of travel restrictions, airport closures, nationwide lockdowns, health risks etc. There is evidence, that incoming tourism agencies in the tourism sector have registered lots of cancellations, decline in the visits and spending of tourists. To give a deep and practical insight related to whatabovementioned, this paper introduces a study on the impact of Covid-19 pandemic situation to the development of inbound tourism in Albania in general, and in specific in the region of Shkodra. The focus of the research was to analyse the challenges faced and changes made in the activity of some incoming tourist agencies in Albania which organize tourist visits in Shkodra region. For this paper was raised the hypothesis “Covid-19 changed the way of offering tourism”. The methodology used was primary research based on a survey completed by a sample of 25 incoming tourist agencies operating in Tirana and Shkodra city which organize tourist visits to Shkodra region, as well as the secondary research based on an analyse of books, journal articles, academic papers, web posts etc. Based on the findings of the study it resulted that incoming tourist agencies, negatively impacted because of pandemic spread, were changing their tourist offer, rearranging their offer by taking measures to be safely adapted considering Covid-19 situation, by making new planning of tourist group size, types of adequate segments, destinations to visit, promotional and price strategies etc. With the progression of vaccination of people, and improvement of the situation, the forecast for the future inbound tourism assume a start and gradual recovery albeit slow at first, during the summer and autumn 2021 with visits to friends and relatives; holiday visits, business trips.

Ports are primary generators of truck traffic in the United States. Seaport operations will require operational and infrastructure changes to maintain the growth of international cargo operations. Truck trip generation models will provide transportation planners and public agencies with valuable information necessary for prioritizing funds for roadway upgrade projects and port infrastructure modifications. A new methodology is presented that combines backpropagation neural networks (BPN) and time series to forecast inbound and outbound heavy truck movements at seaports. The new method uses vessel freight data to identify which parameters are relevant for use as model input in predicting truck traffic at seaports. The method is successfully applied to five ports in Florida—Miami, Tampa, Palm Beach, Jacksonville, and Everglades—thus demonstrating its transferability. Details are provided for the Port of Everglades. The commodities at this port are classified into tons, barrels, and containers. It was found that the primary factors affecting truck traffic are imported containers, imported tonnage, imported barrels, exported containers, exported tonnage, and the particular weekday of operation. Separate BPN models were developed for inbound and outbound truck traffic at the ports. The new method forecasts that the Port of Everglades will have a 33% increase in average daily inbound heavy trucks and a 30% increase in average daily outbound heavy trucks by 2005 (2000 is the base year). The accuracy of the inbound and the outbound truck models is 93% and 92%, respectively.

Cultural tourism is less seasonal than nature tourism. However, previous studies have mainly compared the tourist flow of scenic spots, and it is still unclear how cultural tourism affects regional tourism seasonality. This study investigated the seasonal patterns by analyzing the monthly inbound tourist flow of the 28 typical Chinese tourist cities from 2001 to 2012, and examined the effect of cultural tourism on weakening seasonality by using the random-response panel Tobit model. It was found that the seasonal patterns of inbound tourists present non-peak, one-peak, two-double, and three-peak regular fluctuations, and also have irregular fluctuations affected by emergencies and festivals. Cultural tourism can weaken the seasonality of regional tourism, while nature tourism products enhance tourism seasonality. Suitable travel times throughout the year and destination reception industry levels, locations, and external connections have a significant impact on the regional tourism seasonality, but climate comfort, foreign direct investment, and simply the number of hotels and international travel agencies are not significant for tourism seasonality.

Emerging non-traditional data (NTD) such as transit agencies' smartcard data and Google's General Transit Feed Specifications (GTFS) have made it easier to unveil the way in which public transit remains relevant, reveal how it facilitates daily mobility, and highlight the way in which different locales across a metropolitan area are connected by public transit. Based on a 24-h period of smartcard data for Brisbane (4 March 2014) allied with GTFS data, we retrieved 205,560 distinct transit riders' trip trajectories by direction (AM/inbound vs. PM/outbound) in Brisbane, Australia. It visualises the trajectories using a waterpark metaphor, in that, like water, people flow downhill.

The field of electronic government (e-government) is gaining prominence in contemporary society, as it has a significant influence on the wider populace within the context of a technologically advanced world. E-government makes use of information and communication technologies (ICTs) at various levels and domains within government agencies and the public sector. ICT reduces manual labour, potential fraud points, errors, and process lapses. The Internet’s quick accessibility and the widespread adoption of modern technologies and disciplines, such as big data, the Internet of Things, machine learning, and artificial intelligence, have accelerated the need for e-government. However, these developments raise a number of data reliability and precision concerns. The adoption of blockchain technology by researchers demonstrates its efficacy in addressing such issues. The present study proposes the SECHash system model, which integrates blockchain and Optical Character Recognition (OCR) technologies for the purpose of regulating the processing of incoming documents by governmental agencies. As a case study to assess the proposed system paradigm, the study uses a document containing incoming invoices. The proposal seeks to maintain the integrity of document data by prohibiting its modification after acceptance. Additionally, SECHash guarantees that accepted documents will not be destroyed or lost. The analysis demonstrates that using the SECHash model system will decrease fraudulent transactions by eradicating manual labour and storing documents on a blockchain network.

The relationship between COVID-19 and the tourism industry has important lessons for the post-pandemic period. The tourism industry is undergoing major changes after the pandemic. Analyzing the impact of tourism on the spread of coronavirus around the world may help us to understand how it could be a catalyst for spreading epidemics. To investigate the impact of the tourism industry on the spread of coronavirus, tourism data, as well as cases of coronavirus in the year 2020–2021, were used for OECD countries. The quantile regression method was used to estimate the effects of different types of tourism on the spread of coronavirus. The results showed that, in the first season of 2020, all types of tourists had an impact on the spread of the coronavirus. However, until the end of 2020, only outbound tourism had a significant impact on total deaths caused by the coronavirus, and in 2021, the tourism industry did not have any significant effect on the total deaths caused by the coronavirus. The findings of this article show that prior preparedness, comprehensive guidelines and roadmaps, and the establishment of international travel monitoring agencies are required to assess global constraints in critical situations. Advanced systems for controlling domestic travel in a country at a time of contagious diseases are essential.

Em uma sociedade em que se observa, ao mesmo tempo, agentes que produzem e consomem informação, o intento de obter destaque em meio a imensidão informacional tende a ser uma tarefa desafiadora. Nesse contexto, as empresas têm utilizado os canais digitais para atingir novos consumidores, além de fidelizar clientes antigos. Por essa razão, o presente artigo tem por objetivo promover a discussão teórica de conceitos referentes ao inbound marketing, a user experience e as mídias sociais à luz de uma ótica da área da Ciência da Informação. Para tal, utilizou-se a pesquisa bibliográfica para levantar uma base teórica para sustentar o debate proposto. Com efeito, as relações conceituais observadas permitiram observar convergências entre os objetos estudados, pois ambos dão importância ao fato de compreender as características dos usuários e projetar experiências adequadas a partir disso, além de explicitar a relevância da temática para o campo da Ciência da Informação, visto que este pode se beneficiar adequadamente com o aperfeiçoamento do conhecimento sobre tais estratégias, acerca da sua atuação em mídias sociais digitais. Conclui-se que existem convergências entre as temáticas analisadas e que estão dentro do compêndio de conhecimento necessário para a atuação presente dos profissionais da Ciência da Informação.

Cette thèse CIFRE, financée par une agence de communication, questionne à travers une perspective critique, les pratiques professionnelles des agences françaises de communication digitale, notamment celles utilisant les outils et méthodes Inbound. Nous nous sommes intéressés plus particulièrement, à la manière dont elles mobilisent la conception centrée utilisateur : une notion qu’elles revendiquent et légitiment à travers leurs postures énonciatives. Pour poser les bases de notre réflexion, nous nous sommes d’abord intéressés à trois types de travaux en Sciences de l’Information et de la Communication. Le premier questionne les logiques socio-économiques des agences face aux enjeux du design UX. Quant au deuxième, il relate le processus d’institutionnalisation ou de professionnalisation de la communication, appréhendée comme un outil au service du développement économique de l’organisation. Le troisième interroge enfin, les reconfigurations professionnelles que peut susciter la manipulation d’entités matérielles dans une organisation.Sur le plan méthodologique, nous avons opté pour une démarche ethnométhodologique autour de deux études de cas. Il était question d’aborder le terrain du point de vue des professionnel·le·s évoluant dans deux agences et appréhender leurs pratiques au regard de la conception centrée utilisateur.Les résultats laissent percevoir dans un premier temps, les contours d’un processus de conception très technicisé, guidé par des outils (conçus majoritairement par Hubspot) qui interviennent comme des architextes. Contrairement au travail de légitimation mené par son groupe professionnel (Inbound Marketing France) sur les notions de conception centrée utilisateur, leurs usages participent, à détourner les pratiques de conception vers la « googletisation ». Elle renvoie ici à une activité de conception de supports et actions de communication numérique au cours de laquelle les concepteurs accordent le primat du processus de création aux logiques et préceptes de fonctionnement de Google, au risque d’appauvrir l’expérience vécue par l’utilisateur final. Dans un deuxième temps, nos résultats mettent en exergue la radiographie d’une activité de conception, marquée ou contrainte d’adopter une logique gestionnaire pour répondre à l’impératif d’efficacité marchande des donneurs d’ordre. Cela dit, nous assistons, contre toute attente, aux stratégies de ripostes des acteurs qui ne font pas que subir la position dominante d’un annonceur, à l’affût des moindres fluctuations des courbes de performance des actions et supports de communication. Les acteurs en agence mobilisent des figures et formes rhétoriques dans la mise en disposition des données de performance, y compris dans leurs interprétations. Des êtres extérieurs sont également appelés en renfort par les acteurs en agence, pour apporter une certaine objectivité indiscutable autour de leurs propos. L’usage des formes et figures rhétoriques ne se limite pas uniquement à la disposition et interprétation des données. Il s’invite en amont du processus, notamment dans la répartition des rôles assignés à l’équipe du commanditaire. Il prend la forme un processus de conception où l’équipe du commanditaire endosse sous le regard accompagnateur de l’agence, une posture d’acteur collaborateur à part entière. Selon l’état d’avancement du processus de conception, cette équipe du commanditaire mène à certains endroits, un travail solitaire en formulant dans des matrices à questions, ses représentations de l’usager final. Elle soumet également les solutions capables de faire progresser cet utilisateur final dans le parcours de consommation. Nous avons identifié cette façon de faire des acteurs en agence sous le terme de « rhétorique de la relation de commandite ». Il s’agit en réalité, d’une adaptation de ces agences face au processus d’internalisation des activités de communication mis en place par leurs clients<br>This CIFRE thesis, which is funded by a communication agency, uses a critical perspective to question the professional practices of French digital communication agencies, in particular those using inbound tools and methods. We were more particularly interested in the way they mobilise user-centred design: a notion that they claim and legitimise through their enunciative postures. As the basis of our thinking, we first looked at three types of work in information and communication sciences. The first questions the socio-economic logic of agencies faced with the challenges of UX design. The second relates the process of institutionalising or professionalising communication, which is seen as a tool for the economic development of the organisation. Finally, the third questions professional reconfigurations that can be caused by handling material entities in an organisation.In terms of methodology, we opted for an ethnomethodological approach based on two case studies. It was a question of approaching the issue from the perspective of professionals evolving into two agencies and understanding their practices in relation to the user-centred design.The results initially allow us to perceive the contours of a highly technical design process, guided by tools (mainly designed by Hubspot) which act as architexts. Unlike the legitimation work carried out by its professional group (Inbound Marketing France) on the concepts of user-centred design, their uses are involved in diverting design practices towards “googletisation”. This refers to designing digital communication media and actions during which the designers allow the logical structures and functional precepts of Google to have primacy over the creation process, at the risk of impoverishing the lived experience of the final user. Secondly, our results highlight the radiography of a design activity, which is labelled or forced to adopt a managerial structure to meet the market efficiency imperative of contractors. That said, we are witnessing, against expectations, the response strategies of players who do not merely experience the dominant position of an advertiser on the lookout for the slightest fluctuations in the performance curves of actions and communication media. Actors in agencies use rhetorical figures and forms to provide performance data, including in their interpretations. Third parties are also called in as reinforcements by the actors in the agency, to bring a certain indisputable objectivity to their words. The use of rhetorical forms and figures is not limited to the arrangement and interpretation of data. It is brought in ahead of the process, in particular to distribute the roles assigned to the commissioning team. It takes the form of a design process in which the commissioning team assumes, under the agency's accompanying gaze, the posture of a fully-fledged collaborating actor. Depending on the state of progress of the design process, in some areas this commissioning team has to do the solitary work of formulating its depictions of the end user in question matrices. It also submits solutions capable of advancing this end user along the consumption journey. We have identified this method of agency actors as “sponsorship relationship rhetoric”. In reality, it is an adaptation of these agencies to the process of internalising communication activities set up by their clients

El presente proyecto profesional tiene como objetivo proponer un plan de implementación de la estrategia Inbound marketing que sea adecuado para captar y fidelizar nuevos clientes en el objeto de estudio seleccionado: la agencia de marketing digital Marketing Disruptivo. Por lo mencionado anteriormente, el proyecto inicia con una fase de exploración que tiene como propósito permitirle al lector conocer y comprender a profundidad cómo y dónde inicia la estrategia del Inbound marketing. En línea con ello, se presenta un marco teórico estructurado de tal manera que presenta los conceptos claves del marketing para que, en los siguientes apartados, se continúe con las definiciones y características del marketing digital, el cual abre paso a las estrategias digitales entre las que destaca la de Inbound marketing; con el Inbound marketing inicia la fase descriptiva en donde el proyecto trata de especificar las características y propiedades más importantes de la estrategia, asimismo se inicia la recolección de datos que fue realizada a cuatro grupos diferentes de participantes para llegar a la información indicada y con el mayor detalle posible. Por otro lado, se realizó un análisis de la situación actual del mercado de agencias digitales y del objeto de estudio, Marketing Disruptivo, con la finalidad de presentar el estado actual del entorno en el que la empresa realiza sus actividades y de conocer las necesidades y falencias de la misma. A través de este análisis, y con la información que fue recolectada anteriormente, y con el análisis de cuatro propuestas de implementación de Inbound en una empresa (HubSpot, Inboundcycle, Impulse y GROU Crecimiento Digital), fue posible la presentación de un plan de implementación hecho a través de una elaboración propia basado en las necesidades y fortalezas específicas de la empresa. Como resultado del proyecto, se detallan los pasos que debe seguir la empresa para la aplicación de la propuesta de implementación de la estrategia Inbound marketing en donde cada paso es explicado a partir de los hallazgos obtenidos dentro de las fases de análisis del mercado y recolección de datos. Finalmente, se presentan las conclusiones, las cuales han sido redactadas como producto de la presentación de todo el proyecto profesional y del cumplimiento de los objetivos propuestos en el mismo, y las recomendaciones que se le brinda a Marketing Disruptivo para optimizar sus operaciones.<br>Trabajo de suficiencia profesional

O turismo tem sido reconhecido como um importante indutor de geração de renda e emprego, nesta atividade, a maioria dos empresários administram pequenas e médias empresas, portanto, a escolha de trabalhar em rede tem se mostrado eficiente quando isso acontece de forma organizada. O objetivo dessa pesquisa foi analisar a participação das agências de turismo receptivo como agente fomentador de redes de cooperação no Circuito das Frutas, localizada no Estado de São Paulo. Tal Circuito é formado por 10 municípios: Atibaia, Indaiatuba, Itatiba, Itupeva, Jarinu, Jundiaí, Louveira, Morungaba, Valinhos e Vinhedo. Trata-se de uma pesquisa descritiva exploratória que investigou a relação entre diversos atores da cadeia produtiva do turismo, com apoio em pesquisa documental e bibliográfica. O roteiro de entrevista foi elaborado com base em Hastenreiter Fo. (2005), que estabelece elementos que indicam características de organizações de suporte de rede (OSR), e as análises apoiaram-se em uma matriz, resultante da compilação do referencial teórico, que define categorias e variáveis essenciais para a caracterização de uma rede de cooperação eficiente. Os resultados apresentam que, no caso específico do Circuito das Frutas, as agências de turismo receptivo podem, de fato, fomentar uma rede de cooperação, desde que os objetivos de determinada rede estejam claros, o comprometimento e colaboração dos integrantes sejam fortalecidos e incentivados, as vantagens em participar sejam apresentadas e avaliadas continuamente, desde que haja planejamento e dedicação exclusiva de um gestor responsável em manter os associados interessados em discutir ações comuns e que possa orientar as atividades para os objetivos comuns da rede<br>Tourism has been recognized as a significant job and income fomenter, in this activity, most entrepreneurs manage small and medium-sized companies, thus, choosing to work as a net has been seen as efficient when it happens in an organized manner. This researchs aim was to analyze the participation of inbound tourism agencies as fomenting agents of the cooperation networks in the Fruit Circuit located in the State of São Paulo. This Circuit is comprised by 10 municipalities: Atibaia, Indaiatuba, Itatiba, Itupeva, Jarinu, Jundiaí, Louveira, Morungaba, Valinhos, and Vinhedo. It is an exploratory and descriptive research that investigated the relation among many actors in the tourism chain, based on document and bibliographical research. The interview script was developed based on Hastenreiter Fo (2005), who stablishes elements indicating characteristics of network support organizations (NSO), and the analysis were also based on a matrix that resulted from information in the theoretical references, which defines categories and variables that were considered essential for the characterization of an efficient cooperation network. The outcomes revealed that, in the specific case of the Fruit Circuit, tourism agencies can foment a cooperation network, given that this networks objectives be clear, the members commitment and collaboration be reinforced and encouraged, that the advantages of participating be constantly presented and assessed, and given that there is planning and exclusive commitment by a manager that takes responsibility in keeping associates interested, discussing common actions and guiding activities for the networks\'common objectives

Los trastornos lisosomales y la aciduria glutárica tipo I son las enfermedades objeto de este estudio. Dichas enfermedades, pertenecen al numeroso grupo de enfermedades metabólicas hereditarias (EMH) y tienen en común la deficiencia de una enzima y la consiguiente acumulación del sustrato que al no poderse metabolizar resulta tóxico para la célula. En general, estas enfermedades presentan afectación neurológica, debutan en los primeros años de vida y son devastadoras, tanto para el paciente como para su entorno familiar. En la actualidad, la mayoría de tratamientos disponibles son paliativos o bien no tienen un efecto significativo sobre la afectación neurológica. En este trabajo nos hemos propuesto desarrollar y aplicar diferentes estrategias terapéuticas, bajo diferentes enfoques moleculares, para poder aportar nuevas herramientas en el tratamiento de estas enfermedades. Para ello se han cribado compuestos de bajo peso molecular capaces de atravesar la barrera hematoencefálica para poder, si procede, tratar la afectación neurológica. Nos hemos centrado en el desarrollo de tres aproximaciones diferentes: el uso de terapias con agentes antioxidantes, el uso de chaperonas farmacológicas y el uso de compuestos activadores de la sobrelectura de codones de terminación prematuros (PTCs). Hemos demostrado que el tratamiento con diferentes antioxidantes (coenzima Qio o un cóctel de tocoferol, ácido lipoico y N-acetilcisteina) es capaz de rescatar ciertos parámetros bioquímicos característicos del síndrome de Sanfilippo en fibroblastos de pacientes con esta enfermedad. Por lo tanto, esta aproximación terapéutica podría mejorar la sintomatología de dichos pacientes. Hemos desarrollado un método de cribado de chaperonas farmacológicas para la aciduria glutárica tipo I, que nos ha permitido identificar un posible hit. Se ha validado su efectividad mediante estudios in vitro y en fibroblastos de pacientes con esta enfermedad y se ha confirmado su eficacia en una de las mutaciones más prevalentes en población española (p.Val400Met). Además, hemos colaborado en la creación y validación de un modelo neuronal derivado de células iPS para la enfermedad de Gaucher tipo II (forma neuronopática) y se ha comprobado la efectividad del tratamiento con chaperonas farmacológicas en este nuevo modelo celular mediante el uso de análogos de Nojirimicina. La obtención de estas células iPS permitirá a la comunidad científica tener acceso a un modelo celular neuronal de la enfermedad de Gaucher. Hemos identificado diferentes líneas celulares de pacientes con distintas enfermedades lisosomales, causadas por mutaciones “nonsense”, que responden al tratamiento con compuestos que inducen la sobrelectura de PTCs. En colaboración con otros grupos, hemos puesto a punto un método de cribado de pequeñas moléculas capaces de promover la sobrelectura de PTCs y hemos probado los diferentes hits resultantes en las diferentes líneas celulares previamente seleccionadas. De los más de 62.000 compuestos cribados, únicamente uno demostró restaurar los parámetros bioquímicos alterados en las enfermedades lisosomales de estudio: Bicalutamide. Este compuesto es un anti-androgénico utilizado para el tratamiento de cáncer de próstata que se ha relacionado recientemente con el mecanismo de autofagia. El estudio molecular de su mecanismo de acción nos ha permitido determinar que la restauración de los parámetros bioquímicos alterados después del tratamiento con Bicalutamide era debida a un incremento del flujo autofágico y de la exocitosis lisosomal, determinado por la activación del factor de transcripción TFEB. Este descubrimiento nos ha conducido a la realización de una patente de uso del compuesto Bicalutamide y sus derivados para el tratamiento de enfermedades lisosomales (WO 2015/097088 A1). En resumen, en este trabajo se han desarrollado diferentes estrategias terapéuticas mediante diferentes enfoques moleculares que conforman una primera aproximación para el tratamiento de la afectación neurológica de distintas EMH. Se han identificado dos nuevos compuestos procedentes de librerías de reposicionamiento que han demostrado restaurar parcialmente los parámetros bioquímicos alterados. Estos compuestos podrían ser útiles para el tratamiento de las enfermedades objeto de este estudio, tanto a nivel sistémico como neurológico. Sin embargo, previamente se deberá validar la eficacia de los compuestos identificados en modelos animales.<br>Lysosomal storage disorders (LSDs) and glutaric aciduria type I (AG-I) are inherited metabolic disorders that arise from the deficiency of an enzyme and the subsequent accumulation of its substrate which is harmful for the cell. Most of these diseases present neurological involvement and start during the first years of life being devastating for both, patients and relatives. Nowadays, most of the available treatments are palliative or have no significant effect in the neurological involvement. In this study, we have developed and applied different therapeutic strategies in order to provide new tools for the treatment of these diseases, focusing particularly in targeting the neurological involvement: use of antioxidants, pharmacological chaperones (PCs) and readthrough compounds. We have demonstrated that treatment with antioxidants (coenzyme Qio or a cocktail with tocopherol, lipoic acid and N-acetylcystein) is able to restore the biochemical alterations in fibroblasts derived from Sanfilippo patients. Thus, this therapeutical approach could ameliorate Sanfilippo's patient phenotype. We have developed a high throughput screening methodology to identify possible PCs for the AG-I. We have validated hits effectiveness using in vitro and primary AG-I patient's cell models and confirmed the chaperone activity of one compound in the most prevalent missense mutation in the Spanish population. Moreover, we have validated a neuronal cell model for Gaucher disease created using iPS cells and proved the effectiveness of already described PCs in this cell type, providing a new neurological model for Gaucher disease to the scientific community. We have identified different cell lines derived from patients with nonsense mutations affected by different LSDs responsive to a readthrough treatment. These cell lines have allowed us to validate possible hits resulting from a HTS performed in collaboration with other groups. Only one compound, Bicalutamide, was able to restore the biochemical alterations observed in patients' fibroblasts. Bicalutamide is an anti-androgenic drug used for the treatment of prostate cancer that has been recently related to the autophagy mechanism. Studying its molecular role, we have elucidated that Bicalutamide acts through the activation of the transcription factor TFEB which in turn induces the autophagy and exocytosis fluxes, removing the lysosomal pathogenic accumulation. We have patented its use in LSDs (WO 2015/097088 Al). In conclusion we have developed different therapeutic strategies through different molecular approximations for the treatment of both, the systemic and neurological involvement of the studied diseases. The discovery of these new drugs has to be validated in animals' models.

The thesis deals with the inbound senior tourism. The main aim of the thesis is to analyze the supply of package tours for seniors in the Czech Republic on the basis of the information recorded by the available resources. The other objective is to collect detailed information about senior tourism and to propose appropriate inbound package tours for seniors in the Czech republic on the basis of collected data. The concepts related to the senior tourism and specifics of the senior tourism are defined in the theoretical part of this thesis. The supply of package tours for seniors in the Czech Republic is analyzed in practical part. Web pages of the Czech inbound tour operators and questionnaires in particular are used to collect data. SWOT analysis is used to evaluate current supply. Examples of good practice from both foreign and Czech travel agencies are also mentioned in this thesis. In the conclusion, recommendations about appropriate package tours for seniors are designed on the basis of the primary and secondary data

碩士<br>國立高雄餐旅大學<br>運輸與休閒服務規劃碩士學位學程<br>104<br>With the trend of the global warming, the world is beginning to pay attention to the low carbon economy theory, reducing carbon emissions and realizing the green development. Low-carbon economy emerged against the background of the global warming problem. With the development of a low-carbon economy, the concept of low-carbon tourism is also increasingly popular. Increasing number of studies are focusing on low-carbon tourism and on energy saving and carbon reduction (ESCR) measures. And travel agencies plays an important role, required environmental carbon concept conveyed. Accordingly, in recent years, in the effort of the government, Taiwan has emphasized the development of tourism policies and projects, tourist arrivals in Taiwan have increased to more than 9 million in 2014. The research for inbound travelers as participant to take part in low-carbon tour package of travel agencies, haven't been discussed yet. Therefore, the main purpose of this study is to investigate low-carnon tourism cognition, purchase intention and willing to pay by inbound travelers. In total, 498 valid questionnaires were collected. The research used descriptive analysis, ANOVA analysis, Pearson’s analysis and simultaneous regression to analysis the data. The results of this study indicated that : (1) Low-Carbon tour package has cognitive significantly difference from different backgrounds inbound tourists. (2) Low-Carbon tour package cognitive have a significant impact on the purchase Intention.(3)34.5% of inbound travelers are willing to pay extra 5% regular price for low-carbon tour package ; 32.3% of inbound travelers are willing to pay extra 10% regular price for low-carbon tour package. The results of this study can be provided to government agencies, travel agencies and related sectors as tourism development, as planning low-carbon tour and fixed price reference.

This project was developed in collaboration with a portuguese inbound marketing agency, YouLead, and aims to answer the question “How can YouLead grow in terms of service offering” by defining a strategy that enables the company to grow without losing its positioning as an inbound marketing agency. After analysing the agency industry, YouLead’s resources, capabilities, strengths and weaknesses, as well as the external threats and opportunities, growth hypotheses were discussed. As the market is growing and the agency benefits from a good positioning, the proposed strategy is for YouLead to leverage its strengths and take advantage of market opportunities.

碩士<br>醒吾科技大學<br>觀光休閒系所<br>107<br>"Goods going out, people coming in, everyone makes a fortune." I believe this is the most familiar phrase in 2018. It also tells the people's heart. "Goods going out” means the exportation, “People coming in” means the tourists. Narrowly speaking, it means Mainland tourists, the amount and consuming capacity are both draw international attention. According to the statistics of the International World Tourism Organization and the Tourism Bureau, it can be determined that the tourism market of tourists from mainland China is absolutely important. Furthermore, according to the statistics from the Tourism Bureau, mainland tourists have the largest proportion of shopping expenses during their travels in Taiwan. The huge business opportunities and economic benefits brought by sightseeing and shopping are really not to be underestimated. Shopping is already an indispensable factor in tourism activities. The cooperation between travel agencies and shopping stores are increasing the income outside the business, enhances the price competitive advantage directly. This study also systematically organizes the current situation of Taiwan shopping stores and management regulations. Meanwhile, this study analysis the construction of various industries affected by different commodities which is the purpose to let the government and the people know more about this industry. First, this study establishes the relationship between mainland tourists, Taiwan travel agency and shopping shop, and then determines several items to be discussed. Due to lack of the research of shopping stores in Taiwan, this study uses Deep interview method, Documentation Analysis Method and Secondary Data Collection. The interviewees will be representative of the domestic tourism industry, officials, and academics, who have a certain influence in the industry. The view during the interview will also be classified, and give conclusions and recommendations. Keywords: mainland group tourists（mainland tourists）, inbound travel agencies, shopping stores

With the increase in travel frequency at global level, destinations compete with each other to attract tourists. The tourists have a vast array of destinations to choose from. The destinations compete at global as well as regional level. These developments in tourism bring forth the importance of marketing for a destination. The destination marketing is generally undertaken by destination marketing organizations (DMOs) which operate at national, regional as well as local level in a country. In context of India, MOT (Ministry of Tourism, Government of India) undertakes the role of a DMO and is responsible for formulation and implementation of marketing strategies to promote India as a destination at global level. Further, it is imperative to understand the perception of other stakeholder about the marketing strategies of MOT in order to recognize the effectiveness of its marketing efforts. Being co-creator of destination image, inbound tour operators are considered as the key stakeholders at the destination. The objective of this chapter is to understand the perception Indian inbound tour operators towards the marketing strategies carried out by MOT. The data for the study was collected through structured questionnaire. The findings of the study indicated that inbound tour operators perceived marketing plans adopted by Ministry of tourism were effective to position India as a tourist destination at global level. At planning stage of strategy making for promoting India as tourist destination, MOT does not seek the opinion of inbound tour operators. It is suggested that MOT should involve inbound tour operators for drawing the marketing strategies for India as they are key seller for the tourism product.

Abstract When designing therapeutics for a specific disease, the medicinal chemist must consider the pathological mechanism of the underlying disorder and then design the drug accordingly. For example, anti-neoplastic agents are designed to treat malignancies like colon cancer, whilst anti-inflammatory agents would be designed to treat diseases such as rheumatoid arthritis. Of these ten fundamental pathological mechanisms, most represent errors or imbalances in endogenous mechanisms. Developmental diseases, for example, arise from inborn errors in metabolism or from genetic disorders.

Abstract Fanconi anemia (FA) is a genetically heterogeneous disorder with the clinical features of congenital malformations reminiscent of the VACTERL association, progressive bone-marrow failure, and cancer predisposition. FA cells are characterized by increased sensitivity to DNA cross-linking agents, and FA has thus traditionally been classified as a DNA repair disorder. To date, 13 distinct complementation groups (A, B, C, D1, D2, E, F, G, I, J, L, M, N, and O) have been identified and all of the corresponding genes (FANCA, FANCB, etc.) have been isolated. FA is usually autosomal recessive, but one X-linked form exists. The FANCD1 gene is identical to the familial breast cancer gene BRCA2, and multiple biochemical and genetic interactions of the BRCA1 and BRCA2 genes with FA genes have been found. This has given rise to the concept that the FA and BRCA proteins form a genome maintenance network. This FA/BRCA pathway is activated during normal DNA replication and after DNA damage. The majority of FA proteins interact in a multisubunit FA core complex, which acts to monoubiquitinate the FANCD2 protein. Mono-ubiquitinated FANCD2 interacts with BRCA2 in chromatin to protect cells from replication-associated DNA damage.

Abstract Humans are evolved primates, modeled by the Darwinian agency of natural selection. It is important to understand the nature of this selection. It works always for, and only for, the reproductive success of the individual, the single human, and relatives who can aid the individual by proxy. Selection never works for the good of the group. Pure altruism, giving without return, does not exist. This explains the human paradox. It is in the individual’s interests to be social; but, if threatened, the individual can as readily turn nasty. There is no inborn compulsion to be nice to those who do not reciprocate. Hatred emerges.

Tourism is considered one of the more important industries in the world, contributing economically to many countries’ development (Huang et al., 2008). The Japanese government has actively promoted inbound tourism since the launch of the ‘Visit Japan’ campaign in 2003, by implementing various policies, such as relaxing tourist visas, building a tourist-friendly environment, implementing a tax-free policy and so on. As a result, 28,691,073 international tourists visited Japan in 2017, compared to 5,211,725 in 2003 (JNTO, 2018a, 2018b). Undoubtedly, the booming tourism market brought a significant economic impact to Japan. According to data from the Japan Tourism Agency (JTA, 2017), the economic impact of tourism in 2015 amounted to 25.5 trillion Japanese yen (approximately USD 234 billion), and tourism accounted for four million jobs or 6.7% of nationwide employment (JTA, 2017). However, the tourism industry is also more fragmented and vulnerable to crises and disasters (Faulkner, 2001; Ritchie, 2004), and the industry often finds it difficult to rebound quickly from crises and disasters that have damaged the image of a destination (Cassedy, 1992). Although the number of international tourists to Japan has continually increased since 2003, the industry was greatly affected by the world-wide financial crisis in 2009 and the Great East Japan Earthquake (also called the 3.11 Earthquake and Tohoku Earthquake) in 2011.

Rhonda V. Wilcox’s “Forced Glory: Katniss Everdeen, Bella Swan, and Varieties of Virginity” contrasts Twilight’s Bella Swan and The Hunger Games’ Katniss Everdeen. There are many parallels between the characters, from triumph despite low self-esteem within a first-person narrative to being forced to wear elaborate outfits that serve as signs of power. In early repudiation of marriage and the mother, they reflect the pattern of independence illustrated in Janice Radway’s conceptualization of the romance heroine. Virginity is also central to this pattern, where mental impermeability offers a metaphoric echo. Ultimately, where the characters most differ is in agency. Bella’s protection from (mental) penetration is an inborn ability that helps assimilate her into patriarchy. By contrast, Katniss pretends to have sex while being able to choose virginity. She purposefully and much later chooses procreation, while Bella and Edward assert that in their love, they had no choice.