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Sökning: WFRF:(Maenpaa J)

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  • Watt, Brenda, et al. (författare)
  • Mutations in or near the Transmembrane Domain Alter PMEL Amyloid Formation from Functional to Pathogenic
  • 2011
  • Ingår i: PLoS Genetics. - : Public Library of Science (PLoS). - 1553-7390 .- 1553-7404. ; 7:9, s. e1002286-
  • Tidskriftsartikel (refereegranskat)abstract
    • PMEL is a pigment cell-specific protein that forms physiological amyloid fibrils upon which melanins ultimately deposit in the lumen of the pigment organelle, the melanosome. Whereas hypomorphic PMEL mutations in several species result in a mild pigment dilution that is inherited in a recessive manner, PMEL alleles found in the Dominant white (DW) chicken and Silver horse (HoSi)-which bear mutations that alter the PMEL transmembrane domain (TMD) and that are thus outside the amyloid core-are associated with a striking loss of pigmentation that is inherited in a dominant fashion. Here we show that the DW and HoSi mutations alter PMEL TMD oligomerization and/or association with membranes, with consequent formation of aberrantly packed fibrils. The aberrant fibrils are associated with a loss of pigmentation in cultured melanocytes, suggesting that they inhibit melanin production and/or melanosome integrity. A secondary mutation in the Smoky chicken, which reverts the dominant DW phenotype, prevents the accumulation of PMEL in fibrillogenic compartments and thus averts DW-associated pigment loss; a secondary mutation found in the Dun chicken likely dampens a HoSi-like dominant mutation in a similar manner. We propose that the DW and HoSi mutations alter the normally benign amyloid to a pathogenic form that antagonizes melanosome function, and that the secondary mutations found in the Smoky and Dun chickens revert or dampen pathogenicity by functioning as null alleles, thus preventing the formation of aberrant fibrils. We speculate that PMEL mutations can model the conversion between physiological and pathological amyloid.
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  • Heinola, Ivika, et al. (författare)
  • Open Repair of Mycotic Abdominal Aortic Aneurysms With Biological Grafts : An International Multicenter Study
  • 2018
  • Ingår i: Journal of the American Heart Association. - : John Wiley & Sons. - 2047-9980. ; 7:12
  • Tidskriftsartikel (refereegranskat)abstract
    • Background- The treatment of mycotic abdominal aortic aneurysm requires surgery and antimicrobial therapy. Since prosthetic reconstructions carry a considerable risk of reinfection, biological grafts are noteworthy alternatives. The current study evaluated the durability, infection resistance, and midterm outcome of biological grafts in treatment of mycotic abdominal aortic aneurysm.Methods and Results- All patients treated with biological graft in 6 countries between 2006 and 2016 were included. Primary outcome measures were 30- and 90-day survival, treatment-related mortality, and reinfection rate. Secondary outcome measures were overall mortality and graft patency. Fifty-six patients (46 males) with median age of 69 years (range 35-85) were included. Sixteen patients were immunocompromised (29%), 24 (43%) had concomitant infection, and 12 (21%) presented with rupture. Bacterial culture was isolated from 43 (77%). In-situ aortic reconstruction was performed using autologous femoral veins in 30 patients (54%), xenopericardial tube-grafts in 12 (21%), cryopreserved arterial/venous allografts in 9 (16%), and fresh arterial allografts in 5 (9%) patients. During a median follow-up of 26 months (range 3 weeks-172 months) there were no reinfections and only 3 patients (5%) required assistance with graft patency. Thirty-day survival was 95% (n=53) and 90-day survival was 91% (n=51). Treatment-related mortality was 9% (n=5). Kaplan-Meier estimation of survival at 1 year was 83% (95% confidence interval, 73%-94%) and at 5 years was 71% (52%-89%).Conclusions- Mycotic abdominal aortic aneurysm repair with biological grafts is a durable option for patients fit for surgery presenting an excellent infection resistance and good overall survival.
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  • Sohrabpour, Vahid (författare)
  • Towards satisfying supply chain needs through packaging design and development
  • 2012
  • Licentiatavhandling (övrigt vetenskapligt/konstnärligt)abstract
    • Abstract in UndeterminedPackaging as a component in the supply chain impacts efficiency and effectiveness. In the interaction between packaging systems and the supply chain, actors place various needs on packaging. These can be related to infrastructure or product characteristics, business settings or operations. They also depend on the supply chain context, the distribution conditions, the product and packaging types in use.The purpose of the research presented in this thesis is to bridge the gap between supply chain needs placed on packaging and their satisfaction in the context of developing countries. Three qualitative investigations were conducted. The first was a case study exploring the interactions between the packaging system and the supply chain in developing countries. The second was also a case study about the packaging design and development methods used for fulfilling supply chain needs. The third investigation was a combination of a literature study and interviews to identify various available models and software for corrugated board and box design and to explore their use for different actors in the corrugated board packaging supply chain.Supply chain needs placed on secondary packaging in developing countries are identified, described and categorized. This provides new insights for supply chain managers, logisticians and packaging engineers about the interaction between the supply chain and the packaging system in developing countries. For supply chain managers and logisticians, the information and knowledge about supply chains in developing countries can help them to attain and maintain effectiveness and efficiency in such contexts. For packaging engineers, a generic list of needs can serve as input to packaging design and development methods, processes and analysis tools.The thesis also provides a structured design and development method and an analysis tool that can be used to satisfy the needs placed on packaging in supply chains in developing countries. It offers packaging engineers further insights into how product design and development methods and tools can be adapted for packaging systems to satisfy these needs.The thesis contributes to improving the use and development of prediction models and software for corrugated board and box properties in corrugated board supply chains. Eighteen models and four software programs were investigated and analyzed to provide a basis for choosing the most beneficial ones for different actors in corrugated board supply chains. Eight propositions are provided to improve the use and development of models and software for corrugated board and box in such supply chains. A new holistic perspective is suggested based on a P-diagram that can be used as a theoretical basis for the development and use of prediction models and software for corrugated board packaging.The thesis is multidisciplinary, including supply chain management, packaging design and development, and models and software for predicting properties of corrugated board and boxes. This research mainly contributes to supply chain management, packaging logistics and packaging design and development for the supply chain in the context of developing countries.
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  • Groneck, Max, et al. (författare)
  • Determinants of Relative Sectoral Prices: The Role of Demographic Change
  • 2017
  • Ingår i: Oxford Bulletin of Economics and Statistics. - : Wiley: 24 months. - 1468-0084 .- 0305-9049. ; 79:3, s. 319-347
  • Tidskriftsartikel (refereegranskat)abstract
    • Demographic change raises demand for non-tradable old-age related services relative to tradable commodities. This demand shift increases the relative price of non-tradables and thereby causes real exchange rates to appreciate. We claim that the change in demand affects prices via imperfect intersectoral factor mobility. Using a sample of 15 OECD countries, we estimate a robust increase of relative prices. According to our main estimate, up to one fifth of the average increase in relative prices between 1970 and 2009 can be attributed to population ageing. Further findings confirm the relevance of imperfect factor mobility: Countries with more rigid labour markets experience stronger price effects.
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  • Niemiec, Christopher P., et al. (författare)
  • Self-determination theory and the smoking cessation process : Daily electronic self-reports can identify the initiation of quit attempts
  • 2023
  • Ingår i: Patient Education and Counseling. - Shannon : Elsevier. - 0738-3991 .- 1873-5134. ; 115
  • Tidskriftsartikel (refereegranskat)abstract
    • Objectives: To examine the motivational predictors of the smoking cessation process at the between-persons and within-persons levels.Methods: Mediation analyses were conducted on self-report data (N = 236) that were collected using interval contingent sampling over a 39-day study period.Results: There was a high rate of attrition, as nearly 50% of participants were lost to follow-up. There were credible indirect effects of autonomous self-regulation on smoking behavior on the next day and seven-day abstinence through perceived competence and medication use. At the between-persons level, these models explained 17% of the variance in smoking behavior on the next day and 31% of the variance in seven-day abstinence; at the within-persons level, these estimates were 39% and 57%, respectively.Conclusions: Day-to-day changes in autonomous self-regulation, perceived competence, and medication use are important initiators of the smoking cessation process.Practice implicationsSmokers might be more likely to make a quit attempt if practitioners “tune into” the day-to-day fluctuations of their patients’ motivation for stopping smoking, perhaps using an electronic platform to assess and compare smokers’ current reports to their previous experiences. Such “motivational attunement” can afford practitioners an opportunity to provide need support when patients are willing and able to initiate a quit attempt. © 2023 Elsevier B.V.
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