First dedicated observations of runaway electrons in the COMPASS tokamak

Runaway electrons present an important part of the present efforts in nuclear fusion research with respect to the potential damage of the in-vessel components. The COMPASS tokamak a suitable tool for the studies of runaway electrons, due to its relatively low vacuum safety constraints, high experimental flexibility and the possibility of reaching the H-mode D-shaped plasmas. In this work, results from the first experimental COMPASS campaign dedicated to runaway electrons are presented and discussed in preliminary way. In particular, the first observation of synchrotron radiation and rather interesting raw magnetic data are shown

Assessing the carcinogenic potential of low-dose exposures to chemical mixtures in the environment: the challenge ahead.

Lifestyle factors are responsible for a considerable portion of cancer incidence worldwide, but credible estimates from the World Health Organization and the International Agency for Research on Cancer (IARC) suggest that the fraction of cancers attributable to toxic environmental exposures is between 7% and 19%. To explore the hypothesis that low-dose exposures to mixtures of chemicals in the environment may be combining to contribute to environmental carcinogenesis, we reviewed 11 hallmark phenotypes of cancer, multiple priority target sites for disruption in each area and prototypical chemical disruptors for all targets, this included dose-response characterizations, evidence of low-dose effects and cross-hallmark effects for all targets and chemicals. In total, 85 examples of chemicals were reviewed for actions on key pathways/mechanisms related to carcinogenesis. Only 15% (13/85) were found to have evidence of a dose-response threshold, whereas 59% (50/85) exerted low-dose effects. No dose-response information was found for the remaining 26% (22/85). Our analysis suggests that the cumulative effects of individual (non-carcinogenic) chemicals acting on different pathways, and a variety of related systems, organs, tissues and cells could plausibly conspire to produce carcinogenic synergies. Additional basic research on carcinogenesis and research focused on low-dose effects of chemical mixtures needs to be rigorously pursued before the merits of this hypothesis can be further advanced. However, the structure of the World Health Organization International Programme on Chemical Safety 'Mode of Action' framework should be revisited as it has inherent weaknesses that are not fully aligned with our current understanding of cancer biology

ISPD gene mutations are a common cause of congenital and limb-girdle muscular dystrophies

Dystroglycanopathies are a clinically and genetically diverse group of recessively inherited conditions ranging from the most severe of the congenital muscular dystrophies, Walker-Warburg syndrome, to mild forms of adult-onset limb-girdle muscular dystrophy. Their hallmark is a reduction in the functional glycosylation of α-dystroglycan, which can be detected in muscle biopsies. An important part of this glycosylation is a unique O-mannosylation, essential for the interaction of α-dystroglycan with extracellular matrix proteins such as laminin-α2. Mutations in eight genes coding for proteins in the glycosylation pathway are responsible for ∼50% of dystroglycanopathy cases. Despite multiple efforts using traditional positional cloning, the causative genes for unsolved dystroglycanopathy cases have escaped discovery for several years. In a recent collaborative study, we discovered that loss-of-function recessive mutations in a novel gene, called isoprenoid synthase domain containing (ISPD), are a relatively common cause of Walker-Warburg syndrome. In this article, we report the involvement of the ISPD gene in milder dystroglycanopathy phenotypes ranging from congenital muscular dystrophy to limb-girdle muscular dystrophy and identified allelic ISPD variants in nine cases belonging to seven families. In two ambulant cases, there was evidence of structural brain involvement, whereas in seven, the clinical manifestation was restricted to a dystrophic skeletal muscle phenotype. Although the function of ISPD in mammals is not yet known, mutations in this gene clearly lead to a reduction in the functional glycosylation of α-dystroglycan, which not only causes the severe Walker-Warburg syndrome but is also a common cause of the milder forms of dystroglycanopathy

Data publication: Tailoring pulsed laser deposition fabricated copper oxide film by controlling plasma parameters

Positron annihilation lifetime spectroscopy data from beamtime at ELB

Long slide-away discharges in the COMPASS tokamak

In this contribution, long runaway electron (RE)dominated discharges achieved in the COMPASS tokamak are presented. The extensivelength is possible due to a low consumption of available volt-seconds of thetokamak transformer in this type of discharge. Energetic electron losses in thisregime seems to be modulated mainly by small oscillations of a radial position (controllersetting) unlike in the RE discharges at higher electron density, where variousMHD phenomena affect the evolution of the losses. The behaviour of the slide-awayplasma is studied using magnetic coils, HXR detectors, ECE system and a pair of3He proportional counters of neutrons. The plasma scenario is also modelled usingFokker-Planck codes