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Sanfilippo syndrome type C (mucopolysaccharidosis IIIC) is an early-onset neurodegenerative lysosomal storage disorder, which is currently untreatable. The vast majority of studies focusing on disease mechanisms of Sanfilippo syndrome were performed on non-neural cells or mouse models, which present obvious limitations. Induced pluripotent stem cells (iPSCs) are an efficient way to model human dis

The use of Commercial-off-the-Shelf (COTS) software has become more and more important in state-of-the-art and state-of-the-practice software and system development. The use of COTS software promises faster time-to-market, reduced development cost, increased productivity, and the possibility for companies to focus on their own core competencies. At the same time COTS software raises risks such as

Only during the last two decades has the discourse on evaluation been on the Swedish agenda of social work and social care. One assumption is that a conscious need for evaluation knowledge and evaluation capacity has finally surfaced. This based on the view that work efforts of social service organizations are regarded as chiefly authority-based and to a lesser degree based upon scientific knowled

In this paper, we discuss research problems for enabling content distribution and supporting real-time interpersonal communication services (e.g. voice and video) over best effort networks with sufficient quality. We take a practical view of content distribution and quality, and this is the reason for the term “sufficient”. We argue that the understanding of quality as perceived by the user is a k

Abstract in UndeterminedA more or less continuous process of structural change, not least due to a globalizing economy, poses a number of challenges to regions and local communities. However, when firms or whole industries are phasing out, windows of opportunity open up. Empirical analyses of two recent plant closures in Skane point at the importance of retaining existing knowledge bases, and at t

It is frequently observed that the local relative abundances of aquatic microbial taxa are correlated with their average relative abundance at the regional scale, which results in the composition of different communities being more similar than expected by chance or invariant. The degree to which communities within a region match the regional average community is variable and likely depends on sev

The design and fabrication of a home-made thin-layer electrochemical flow cell are reported. The performance and electrochemical characteristics of thin-layer cells are evaluated in terms of flow rate and channel thickness. The adaptability of the flow cell for amperometric and electrochemiluminescence measurements is demonstrated. Also, a flow injection method comprising electrochemical generatio

It has been suggested that organic farming may benefit farmland biodiversity more in landscapes that have lost a significant part of its former landscape heterogeneity. We tested this hypothesis by comparing bird species richness and abundance during the breeding season in organic and conventional farms, matched to eliminate all differences not directly linked to the farming practice, situated in

There is an increasing interest in achieving gene regulation in biotechnological and biomedical applications by using synthetic DNA-binding agents. Most studies have so far focused on synthetic sequence-specific DNA-binding agents. Such approaches are relatively complicated and cost intensive and their level of sophistication is not always required, in particular for biotechnological application.

Partially Premixed Combustion is a concept able to combine low smoke and NOx emissions with high combustion controllability and efficiency. It is of interest to be able to utilize PPC in a large operating region in order to meet the Euro VI emission legislation without relying on NOx aftertreatment. This paper investigates the differences in PPC characteristics for three fuels; Diesel Swedish Mk

The price of fish depends on quality attributes such as size and freshness. In turn, quality attributes are related to fishery management. This article presents a hedonic analysis where attribute prices of size and quality ratings are estimated for the Swedish Baltic cod fishery. Using information from 5,307 landing days, hedonic inverse demand functions are estimated with a random coefficient (RC

The problems of allocation of scarce resources and priority setting in health care have so far not been much studied in the context of stem cell-based therapeutic applications. If and when competitive cost-effective stem cell-based therapies are available, the problem of priority setting - to whom should stem cell-based therapies be offered and on what grounds - is discussed in this article using

Development and application of some laser techniques for the study of engine combustion are presented. The investigations mainly cover two aspects: fuel dynamics and formation of NO. Fuel dynamics is one of the most central problems in engine design since many performance parameters are affected by it; emission formation, efficiency, drivability etc. The studies include cycle-resolved measurement