Thymidine and deoxyuridine accumulate in tissues of patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE).

AbstractMitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disease due to ECGF1 gene mutations causing thymidine phosphorylase (TP) deficiency. Analysis of post-mortem samples of five MNGIE patients and two controls, revealed TP activity in all control tissues, but not in MNGIE samples. Converse to TP activity, thymidine and deoxyuridine were absent in control samples, but present in all tissues of MNGIE patients. Concentrations of both nucleosides in the tissues were generally higher than those observed in plasma of MNGIE patients. Our observations indicate that in the absence of TP activity, tissues accumulate nucleosides, which are excreted into plasma

Seismic isolation of the IRIS nuclear plant

The safety-by-design™ approach adopted for the design of the International Reactor Innovative and Secure (IRIS) resulted in the elimination by design of some of the main accident scenarios classically applicable to Pressurized Water Reactors (PWR) and to the reduction of either consequences or frequency of the remaining classical at-power accident initiators. As a result of such strategy the Core Damage Frequency (CDF) from at-power internal initiating events was reduced to the 10-8/ry order of magnitude, thus elevating CDF from external events (seismic above all) to an even more significant contributor than what currently experienced in the existing PWR fleet. The same safety-by-design™ approach was then exported from the design of the IRIS reactor and of its safety systems to the design of the IRIS Nuclear Steam Supply System (NSSS) building, with the goal of reducing the impact of seismically induced scenarios. The small footprint of the IRIS NSSS building, which includes all Engineered Safety Features (ESF), all the emergency heat sink and all the required support systems makes the idea of seismic isolation of the entire nuclear island a relatively easy and economically competitive solution. The seismically isolated IRIS NSSS building dramatically reduces the seismic excitation perceived by the reactor vessel, the Containment structure and all the main IRIS ESF components, thus virtually eliminating the seismic-induced CDF. This solution is also contributing to the standardization of the IRIS plant, with a single design compatible with a variety of sites covering a wide spectrum of seismic conditions. The conceptual IRIS seismic isolation system is herein presented, along with a selection of the preliminary seismic analyses confirming the drastic reduction of the seismic excitation to the IRIS NSSS building. Along with the adoption of the seismic isolation system, a more refined approach to the computation of the fragility analysis of the components is also being developed, in order to reduce the undue conservatism historically affecting seismic analysis. The new fragility analysis methodology will be particularly focused on the analysis of the isolators themselves, which will now be the limiting components in the evaluation of the overall seismic induced CDF. Copyright © 2009 by ASME

Early watermills – an archaeological indication of taxation?

The introduction of watermills in Southern Scandinavia has often been linked to the advent of the Cistercian Order and regarded a kick-starter for the so-called medieval revolution. In the present article, the archaeological evidence for watermills predating the religious order will be investigated and an earlier and alternative origin laid out. Here, the increased specialisation and centralisation pertaining to the Late Iron Age and Viking Age will be introduced as a significant cause for the initial construction of watermills, and the extensive excavations at Viking Age Omgaard, Denmark, will figure as case in point. Also, essential social mechanisms such as taxation and elite privileges will be highlighted as overlooked triggers in the Viking Age employment of watermills