Pompe disease is a progressive metabolic myopathy caused by deficiency in lysosomal acid α-glucosidase and results in cellular lysosomal and cytoplasmic glycogen accumulation. A wide spectrum of clinical phenotypes exists from hypotonia and severe cardiac hypertrophy in the first few months of life to a milder form with the onset of symptoms in adulthood. The disease is typically due to severe mut

Cytochrome c oxidase encoded by multiple mitochondrial genes (COXI, COXII, and COXIII) and nuclear genes is an essential component of the mitochondrial respiratory chain that catalyzes the reduction of molecular oxygen by reduced cytochrome c. Subunits COXI and COXII of cytochrome c oxidase are known to play the most essential role in proton pumping and electron transfer. In this study we screened

Street lighting is an essential feature of pedestrian paths that may increase the amount of walking. This study aimed to identify associations between perceived lighting qualities, and experience of the lit environment and performance of critical tasks. In a full-scale laboratory 89 participants walked along a pedestrian path with three different outdoor lighting applications (CDM and LEDs). After

To facilitate the justification of an X-ray or nuclear medicine investigation and for informing patients, it is desirable that the individual patient's radiation dose and potential cancer risk can be prospectively assessed and documented. The current dose-reporting is based on effective dose, which ignores body size and does not reflect the strong dependence of risk on the age at exposure. Risk es

Are capitalists more likely than others to justify ‘immoral’ acts such as bribery ortax evasion? This chapter investigates the conditions under which this is the case.While qualitative studies continue to document the nuances, mechanisms, andpractices of cheating and fraud among capitalists, quantitative work that tests the presence of fraud in modern economic life across a wide range of societies

How, in historical societies, did people finalise transactions? Over the past few decades many economic and social historians have concerned themselves with this question, following the examples set by Douglass North and Craig Muldrew. Surprisingly, they have almost completely disregarded the most straightforward solution that historical societies had to offer, namely by using coins and currencies

Our article aims to detect differences in behaviour towards the capital and land market of households of different sizes and levels of income. Participation in markets for real estate, capital, and ships was considerable, not only among middling and elite groups but also among the poor. Large households were most active in markets, incurring significantly greater debt. Poor households with a low s

Methane is produced usually from organic waste in a straightforward anaerobic digestion process. However, hydrogen production is technically more challenging as more stages are needed to convert all biomass to hydrogen because of thermodynamic constraints. Nevertheless, the benefit of hydrogen is that it can be produced, both biologically and thermochemically, in more than one way from either orga

: Current practice of sustainable design encompasses a range of green strategies. Modernpassive design concepts have learned to build upon climate responsive methodology that was found in andinspired from vernacular and traditional buildings. With the present energy crises in Egypt there is a currentfocus on the development of climate sensitive design building approaches. It will be the thrust of

A general framework for analysing robust synchronization in large-scale heterogenous networks is proposed based on the theory of integral quadratic constraints (IQCs). Dynamic agents are represented as linear time-invariant single-input-single-output systems. The agents exchange information according to a sparse dynamical interconnection operator in order to achieve synchronization, where their ou

To keep the spatially averaged temperature and thermal stress of gas turbine blades and guide vanes within a permissible level, the present research concentrates on the optimization of the internal cooling passages in a guide vane. The main purpose is to search for the most optimal sizes, distributions, and shapes of internal cooling channels located in a guide vane. Cylindrical cooling passages a

Drawing from several years of field work and about 30 life story interviews,this chapter presents a case of contemporary evangelical mobility that cannotbe squarely placed within any of the related academic debates about ‘(religious)tourism’, ‘pilgrimage’, ‘short-term missions’, ‘international volunteering’ or ‘migration’ simply because it is intrinsically related to all these phenomena at the sam

We present a spectral and inverse spectral theory for the zero dispersion spectral problem associated with the Camassa-Holm equation. This is an alternative approach to that in [10] by Eckhardt and Teschl.

Forest degradation, forest fires, and wildlife poaching have devastated biodiversity in Indonesia. To assess the impact of forest degradation and the potential for recovery, we used birds as a proxy for biodiversity and assessed density estimates (hereafter density) in the degraded lowland forest of Harapan Rainforest Ecosystem Restoration Concession (HRF) in Sumatra. In this study, a total of 149