Background: Focal cortical dysplasias (FCDs) are congenital structural abnormalities of the brain, and represent the most common cause of medication-resistant focal epilepsy in children and adults. Recent studies have shown that somatic mutations (i.e. mutations arising in the embryo) in mTOR pathway genes underlie some FCD cases. Specific therapies targeting the mTOR pathway are available. However, testing for somatic mTOR pathway mutations in FCD tissue is not performed on a clinical basis, and the contribution of such mutations to the pathogenesis of FCD remains unknown. Aim: To investigate the feasibility of screening for somatic mutations in resected FCD tissue and determine the proportion and spatial distribution of FCDs which are due to low-level somatic mTOR pathway mutations. Methods: We performed ultra-deep sequencing of 13 mTOR pathway genes using a custom HaloPlexHS target enrichment kit (Agilent Technologies) in 16 resected histologically-confirmed FCD specimens. Results: We identified causal variants in 62.5% (10/16) of patients at an alternate allele frequency of 0.75–33.7%. The spatial mutation frequency correlated with the FCD lesion’s size and severity. Conclusions: Screening FCD tissue using a custom panel results in a high yield, and should be considered clinically given the important potential implications regarding surgical resection, medical management and genetic counselling.

To meet the international biodiversity targets of the 2011–2020 Global Strategy for Plant Conservation, it is important to assess the success of coordinated ex situ plant conservation initiatives such as the European Native Seed Conservation Network (ENSCONET), which operated during 2005–2009, and the ENSCONET Consortium, which was established in 2010. In particular, analysis of the ENSCONET database (ENSCOBASE) indicates that ex situ seed banks have been making significant progress towards meeting targets 8 (at least 75% of threatened plant species in ex situ collections, preferably in the country of origin, and at least 20% available for recovery and restoration programmes) and 9 (70% of the genetic diversity of crops, including their wild relatives and other socio-economically valuable plant species, conserved, while respecting, preserving and maintaining associated indigenous and local knowledge) for native European species. However, the infraspecific diversity of threatened species stored in ENSCONET seed banks needs to be increased to meet research and conservation objectives.

The tetracyclines (TTC) and sulfonamides are among the most common residues found in bulk raw milk samples. Detection of drug residues in bulk milk (BM) tankers demonstrates that the product is not suitable for human consumption. Discarding BM with residue-contaminated milk is a waste of a valuable commodity, and a repurposing for consumption at calf ranches is a way to recapture some value. However, if calves consuming milk with drug residues are slaughtered for veal, their meat could contain drug residues. The objective of this review is to provide a residue avoidance strategy for TTC and sulfonamide residues in veal. To determine the pharmacokinetic properties of each drug a structured review of the literature was performed and the study inclusion criteria were that the publication used dairy breed calves, with body weight <330 kg or <6 months of age. The most pertinent parameters were determined to be plasma, tissue elimination half-lives, and systemic bioavailability. The results of this review were integrated with milk and tissue testing levels of quantification and tissue tolerances to formulate a recommended withdrawal interval for calves ingesting this milk. The suggested withdrawal interval of 20 days will ensure that no veal calves will test positive for residues from being fed this milk.

The safety of the food supply is a subject of intense interest to consumers, particularly as a result of large-scale outbreaks that involve hundreds and sometimes thousands of consumers. During the last decade, this concern about food safety has expanded to include the diets of companion animals as a result of several incidences of chemical toxicities and infectious disease transmission. This has led to increased research into the causes and controls for these hazards for both companion animals and their owners. The following summary provides an introduction to the issues, challenges and new tools being developed to ensure that commercial pet foods are both nutritious and safe.

The 2006 Highly Pathogenic Avian Influenza (HPAI) outbreak in Egypt saw the adoption of a fierce stamping-out policy with the culling of 30 million birds in a matter of weeks. This was coupled with an ad hoc compensation scheme that led to wide misuse and rapid depletion of allocated funds. Since September 2006, no compensation has been paid.

HPAI in Egypt is now believed to be endemic and a comprehensive, transparent and fair compensation policy is needed to encourage disease reporting. With or without compensation, rehabilitation of the poultry producing units will occur. Strong veterinary engagement in the start up activities of small producers could be a means to improve biosecurity and establish trust. This paper outlines FAO's activities related to an extensive exercise undertaken to support the government of Egypt in formulating and implementing a compensation policy and strategy, which ensures that poor backyard poultry producers (usually women) are fairly compensated; and an investigation into how smallholder poultry producer rehabilitation activities are currently operating and how these activities can be supported.

La mesure de la radioactivité atmosphérique constitue une difficulté métrologique majeure. En fonction des mécanismes de prélèvement sur filtre et des paramètres de mesure, l'activité alpha des aérosols est sous estimée. L’énergie cinétique des particules alpha est atténuée dans la matière qu’elles traversent, et une partie de cette énergie ne parvient plus au détecteur. Le rendement de mesure est alors dégradé et nécessite une correction. Pour répondre aux conditions de mesures usuelles, le laboratoire a développé un modèle empirique pour l'évaluation des corrections de l’activité alpha en fonction de la masse des aérosols déposés sur le filtre. Le modèle a été validé expérimentalement et a permis une meilleure estimation de l'incertitude sur la mesure de l'activité alpha des aérosols. L'étude a contribué à une première évaluation du facteur de correction pour des filtres peu encrassés provenant de la région parisienne : Fαlabo= 1,6 ± 0,5.

Plasma-assisted nitriding is an attractive surface treatment for metallurgical surface modification to improve wear, hardness and fatigue resistance of austenitic stainless steels. However, this technique requires low temperature processing in order to avoid chromium nitride precipitation and hence the degradation of corrosion properties. This paper presents a low temperature high rate plasma nitriding process and will emphasis on the consequences of nitrogen incorporation on the metallographic and crystallographic properties of the sample surface.

Internal oxidized copper was tested by isothermal mechanical spectroscopy in a medium temperature range (300–600 K). Experimental results show the existence of a non-thermally activated effect at low temperature and of a relaxation peak at higher temperatures. The material microstructure was studied by combination of Transmission Electron Microscopy (TEM) and Electron Energy Loss Spectrometry (EELS). The TEM study allowed us to investigate the distribution of fine spherical particles and the presence of particular network dislocations inside the grains. The EELS method was used to identify the nature of these fine particles as Cu2O. The internal friction has revealed a non thermally activated maximum occurring at 0.1 Hz for temperatures ranging from 290 K to 394 K, and a relaxation peak obtained after annealing at 573 K. This peak is stable after successive annealings at 723 K and 873 K. Comparison of the microstructure observations, their evolution with annealing and the evolution of the relaxation effect with annealing temperature enables us to interpret the phenomena described in this work: on the one hand, the microstructural characterisation using TEM and EELS allows us to assign the first effect to the result of a transformation of metastable Cu2O particles to CuO under the cyclic stress; on the other hand, the relaxation peak that does not change after high temperature annealing is linked with a particular stable dislocation network observed in many grains. 


Growth, tolerance and zinc and cadmium hyperaccumulation of Thlaspi caerulescens populations from three metal contaminated soils and three normal soils were compared under controlled conditions. Individuals of six populations were cultivated on five soils with increasing concentrations of zinc (50–25000 μg g−1) and cadmium (1–170 μg g−1). There was no mortality of normal soil populations in the four metal-contaminated soils, but plant growth was reduced to half that of populations from metal-contaminated soils. However, in noncontaminated soil, the growth of individuals from normal soils was greater than that of individuals from metal-contaminated soils. Individuals from normal soils concentrated three times more zinc in the aboveground biomass than those from metal-contaminated soils, but the latter accumulated twice as much cadmium. We conclude that populations of T. caerulescens from both normal and metal-contaminated soils are interesting material for phytoextraction of zinc and cadmium, but to optimize the process of phytoextraction it is necessary to combine the extraction potentials of both type of populations.

The effects of long-term oral administration of enkephalinase inhibitors (acetorphan and thiorphan) on food and water intake, live-weight gain and food conversion efficiency were investigated in growing rats and mice. In rats, daily drenching with acetorphan (an absorbable prodrug of thiorphan) at 1 mg/kg per day for 8 days did not alter food and water consumption but significantly increased live-weight gain (32·0 (s.d. 5·1) g for control rats v. 40·7 (s.d. 9·1) g for treated rats (P ≤ 0·05 U test) and improved food conversion efficiency (4·37 (s.d. 0·49) g food per g gain for control rats v. 3·70 (s.d. 0·67) g food per g gain for treated rats). In mice, lower doses (0·2 mg/kg per day) of thiorphan and acetorphan given in the drinking water similarly affected live-weight gain (7·7 (s.d. 0·9) g v. 6·0 (s.d. 1·6) g in 3-week-old mice receiving thiorphan and 2·6 (s.d. 0·4) g in 5-week-old mice receiving acetorphan) with a likely improvement in food conversion efficiency. These results suggest that oral administration of enkephalinase inhibitors may alter growth in rodents, probably by affecting the digestive process.

We have used x-ray diffraction to measure the strain perpendicular to the substrate surface in laser crystallized silicon films on oxidized silicon and fused quartz substrates. The dependence of the strain on grainorientation was determined and the influence of the scan speed, the insulating oxide thickness, and subsequent high temperature exposure was examined. Maximum strain was obtained for grains oriented with the (100) plane parallel to the substrate surface. The strain decreased with increasing angle between the surface plane and the (100) plane of the grains. The stress parallel to the surface in the variously oriented grains was calculated from the stiffness tensor, assuming an isotropic, in-plane stress, and a variation similar to the strain was found. The strain found on oxidized wafers was about half that on fused quartz. Its dependence upon the oxide thickness (0.2 μm to 1.0 μm) was not significant for scan speeds under 10 cm/sec. Similarly, the variation in strain with scan speed was very small for speeds below 10 cm/sec. Scan speeds above 50 cm/sec caused significant increases in the strain.

The measured strain was reduced by high temperature anneals. A 1100°C anneal reduced the average strain by 60% and caused a clear reduction in grain imperfections (as determined by diffracted beam width). However, a 900'C anneal increased the diffracted beam widths even though the average strain was reduced by about 30%.

A new approach is developed, employing “in situ” electrical resistivity measurements, as a tool to study ion beam mixing of evaporated metal-metal multi or bilayers. The electrical resistivity variations measured continuously during the ion bombardment exhibit a monotonical increase and a tendency toward a saturation process allowing to detect precisely the total mixing of the film. The volume fraction of intermixed atoms can be determined within the framework of a simple conduction model. Experimental results are given in the case of Fe-Al and Al-Ag multilayers.

Evaporated bilayers and multilayers of Fe and Al have been studied during ion beam mixing with Xe ions using in-situ electrical resistivity measurements. Experiments have been performed in the composition range 40 – 58 at.% Al and at both temperatures 77 K and 300 K. A semi-empirical model is proposed to explain the observed kinetics. At low doses, a square root dependence of the mixed volume fraction on dose is found at 77 K but not at 300 K. The results are discussed by comparison with the different models proposed for ion beam mixing.

We present an experimental method, based on the isothermal nature of the boundaries of explosively crystallized silicon, to display instantaneous isotherms produced by fast scanning laser beams. This method is especially useful for the case of multilayer structures or complex geometries, where thermal profiles simulation is a difficult problem. We can also obtain directly a measure of the spatial intensity distribution in the laser beam.

Two b.c.c. ordered alloys Fe-40 at % Al and Ni-Ti have been investigated by transmission electron microscopy after self ion implantation Al+ and Ni+ respectively. Partially disordered regions are observed in the Fe-40 at % Al. On the contrary, in the Ni-Ti alloy, complete transformation to the amorphous state occured. During post implantation annealings, the precipitation of a new f.c.c. phase is observed in the Fe-40 at % Al ordered alloy, in the amorphous Ni-Ti the recrystallization occurs in a temperature range 400 – 550°C; the new phase has a structure either martensite type or B2 type with modulatiens.