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Introduction: In 2011, a homozygous mutation in GOSR2 (c.430G > T; p. Gly144Trp) was reported as a novel cause of Progressive Myoclonus Epilepsy (PME) with early-onset ataxia. Interestingly, the ancestors of patients originate from countries bound to the North Sea, hence the condition was termed North Sea PME (NSPME). Until now, only 20 patients have been reported in literature. Here, we provide a
Postscript to a small volume with poems by Hungarian poet Daniel Dányi (b. 1981)
En central del av många internetföretags verksamhet är insamlingen, lagringen och användningen av personuppgifter som överlämnats till bolagen genom registrering. Med personuppgifter avses all information som kan hänföras till en specifik person och i takt med samhällets globalisering har denna information kommit att få ett betydande kommersiellt värde. Överföringen av personuppgifter från EU tillTo various internet companies the collection, storage and use of personal data that has been transmitted through registration is an essential part of the companies business. Personal data refers to all information relating to a specific person and as a consequence of society’s globalization this information has a considerable commercial value. The transfer of personal data from the EU to the US is
This thesis analyses the impact of social networks on technology adoption among smallholder farmers in Ethiopia. The effects of four types of social networks on adoption of fertilizer and soil conservation practices were estimated using a sample of households from the Ethiopian Rural Household Surveys (ERHS). Trusted kinship links were found to have a small but positive effect on fertilizer adopti
The increase in global competitiveness and the advancement of digital technology are compelling organizations to innovate by enhancing their product offerings with digital services. Although this phenomenon known as digital servitization is well researched in larger organizations, there is less investigation on how small and medium enterprises (SMEs) manage this trend, particularly in Sweden. This
This thesis explores how entrepreneurs experience and regulate fluctuations in passion, and how these affect their decision-making in high-stakes contexts. While entrepreneurial passion has been widely recognised as a motivating force, little is known about how its intensity fluctuates over time and how such shifts influence strategic behaviour and decisions. Drawing on emotion regulation theory,
Brazil’s National Strategy for the Impact Economy (ENIMPACTO), launched in 2017, is a public initiative that fosters an ecosystem where businesses combine financial returns with positive social and environmental outcomes. While the strategy has earned both national and international recognition, its implementation and the role of intermediary organizations in this process remain underexplored in a
Conventional agriculture, dominated by annual crops, has revolutionised food production but also introduced significant environmental and social challenges. Perennial crops offer a promising alternative, with potential benefits for ecosystem services, agricultural sustainability, and food security. Although the environmental benefits of perennial crops, such as the intermediate wheat grass Kernza
There is scientific consensus that limiting warming in line with the Paris Agreement goals requires reaching net zero CO2 emissions by mid-century and net negative emissions thereafter. Because of the entrenchment of current fossil fuel energy and feedstock demand estimated in almost all global modelled scenarios, 'abated' fossil fuel and industrial process and product use (IPPU) CO2 emissions, us
Mali and Somalia have both suffered determined Islamist-inspired insurgencies, and in both African Union-led peace operations have been a central pillar in political and security stabilization efforts. Despite challenges in transferring lessons between unique situations, the AMISOM experience can offer some useful lessons for Mali. We have identified several themes that helped to drive success for
This study aimed to determine urine pesticide metabolite concentrations using liquid chromatography and investigate associated exposure factors in a rural area of Bogotá, Colombia. In 2019, 231 urine samples were collected from children aged 7 to 10. Urinary pesticide metabolite concentrations were measured, and a questionnaire was administered to the children’s mothers to collect exposure-related
This study examined how root growth and morphology were affected by variation in soil moisture at four Amazon rainforest sites with contrasting vegetation and soil types. Mean annual site root mass, length and surface area growth ranged between 3-7 t ha(-1), 2-4 km m(-2) and 8-12 m(2) m(-2) respectively. Mean site specific root length and surface area varied between 8-10 km kg(-1) stop and 24-34 m
The unimolecular chemiluminescent decomposition of unsubstituted dioxetanone was studied at the complete active space self-consistent field level of theory combined with the multistate second-order multiconfigurational perturbation theory energy correction. The calculations revealed interesting features. Two transition states, two conical intersections, and one intermediate stable biradical struct
This thesis investigates the restitution of cultural artefacts looted during the colonial-era, through the comparison between two United Nations Educational, Scientific and Cultural Organization (UNESCO) Conventions and the newly proposed French guideline, Shared Heritage: Universality, Restitution and Circulation of Works of Art (SHURCWA). Through a qualitative case study employing Carol Bacchi’s
This paper investigates the determinants of corporate cash holdings in the casino and gambling industry. The trade-off, pecking order and free cash flow theory are all popular methods for explaining the determinants of cash holdings for general firms. This paper examines traditional financial variables, macroeconomic variables and a specific time period variable for the effects of the financial cr
The large number of orphanages in Jinja, Uganda has promoted the widespread institutionalisation of children from poor families. Parents are left disempowered and have very few options but to sign over the rights of their children because NGOs in the region have largely prioritised orphanage care over other services. This paper critically analyses the failure of non-state actors in addressing the
För att undersöka barns fulla förståelse av ords betydelser är det nödvändigt att undersöka förståelse av ord i relation till andra ord. Antonymi, motsatsrelationen, är en av de semantiska relationer som barnet tillägnar sig tidigast i språkutvecklingen. Syftet med studien var att undersöka förskolebarns förmåga till motsatsordsmobilisering, det vill säga förmågan att välja rätt motsatsord som res
The aim of the study is to research preschool teacher’s understanding and way of talking about ethnicity and diversity in two different municipalities. The design of the methods performed for the study was qualitative and seven interviews have been done to gather empirics. The interview guide was semi-structured in order to be able to keep a flexible approach through the interviews. The result sho
