1,276 research outputs found
Conceptual study of an ICRH traveling-wave antenna system for low-coupling conditions as expected in DEMO
For the central heating of a fusion reactor ion cyclotron radio frequency heating (ICRH) is the first choice method as it is able to couple RF power to the ions without density limit. The drawback of this heating method is the problem of excitation of the magneto-sonic wave through the plasma boundary layer from the antenna located along the wall, without exceeding its voltage standoff. The amount of coupling depends on the antenna excitation and the surface admittance at the antenna output due to the plasma profile. The paper deals with the optimization of the antenna excitation by the use of sections of traveling-wave antennas (TWAs) distributed all along the reactor wall between the blanket modules. They are mounted and fed in resonant ring system(s). First, the physics of the coupling of a strap array is studied by simple models and the coupling code ANTITER II. Then, after the study of the basic properties of a TWA section, its feeding problem is solved by hybrids driving them in resonant ring circuit(s). The complete modeling is obtained from the matrices of the TWA sections connected to one of the feeding hybrid(s). The solution is iterated with the coupling code to determine the loading for a reference low-coupling ITER plasma profile. The resulting wave pattern up to the plasma bulk is derived. The proposed system is totally load resilient and allows us to obtain a very selective exciting wave spectrum. A discussion of some practical implementation problems is added
Ion cyclotron resonance heating scenarios for DEMO
International audienceThe present paper offers an overview of the potential of ion cyclotron resonance heating (ICRH) or radio frequency (RF) heating for the DEMO machine. It is found that various suitable heating schemes are available. Similar to ITER and in view of the limited bandwidth of about 10M Hz that can be achieved to ensure optimal functioning of the launcher, it is proposed to make core second harmonic tritium heating the key ion heating scheme, assisted by fundamental cyclotron heating 3 He in the early phase of the discharge; for the present design of DEMO-with a static magnetic field strength of B o = 5.855T-that places the T and 3 He layers in the core for f = 60M Hz and suggests to center the bandwidth around that main operating frequency. In line with earlier studies for hot, dense plasmas in large-size magnetic confinement machines it is shown that good single pass absorption is achieved but that the size as well as operating density and temperature of the machine cause the electrons to absorb a non-negligible fraction of the power away from the core when core ion heating is aimed at. Current drive and alternative heating options are briefly discussed and a dedicated computation is done for the traveling wave antenna, proposed for DEMO in view of its compatibility with substantial antenna-plasma distances. The various tasks that ICRH can fulfill are briefly listed. Finally, the impact of transport and the sensitivity of the obtained results to changes in the machine parameters is commented on
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Recent modeling for the ITER ion cyclotron range of frequency antennas with the TOPICA code
This paper documents the analysis of the ITER ion cyclotron resonance heating (ICRF) launcher using the TOPICA code, throughout recent years' design activities. The ability to simulate the detailed geometry of an ICRF antenna in front of a realistic plasma and to obtain the antenna input parameters, the electric currents on conductors and the radiated field distribution next to the antenna is of significant importance to evaluate and predict the overall system performances. Starting from a reference geometry, we first investigated the impact of some geometrical and numerical factors, such as the Faraday Screen geometry or the mesh quality. Then a final geometry was the object of a comprehensive analysis, varying the working frequency, the plasma conditions and the poloidal and toroidal phasings between the feeding lines. The performance of the antenna has been documented in terms of input parameters, power coupled to plasma and electric fields. Eventually, the four-port junction has also been included in TOPICA models
High Incidence of Noonan Syndrome Features Including Short Stature and Pulmonic Stenosis in Patients carrying NF1 Missense Mutations Affecting p.Arg1809: Genotype-Phenotype Correlation
Neurofibromatosis type 1 (NF1) is one of the most frequent genetic disorders, affecting 1:3,000 worldwide. Identification of genotype-phenotype correlations is challenging because of the wide range clinical variability, the progressive nature of the disorder, and extreme diversity of the mutational spectrum. We report 136 individuals with a distinct phenotype carrying one of five different NF1 missense mutations affecting p.Arg1809. Patients presented with multiple cafe-au-lait macules (CALM) with or without freckling and Lisch nodules, but no externally visible plexiform neurofibromas or clear cutaneous neurofibromas were found. About 25% of the individuals had Noonan-like features. Pulmonic stenosis and short stature were significantly more prevalent compared with classic cohorts (P \u3c 0.0001). Developmental delays and/or learning disabilities were reported in over 50% of patients. Melanocytes cultured from a CALM in a segmental NF1-patient showed two different somatic NF1 mutations, p.Arg1809Cys and a multi-exon deletion, providing genetic evidence that p.Arg1809Cys is a loss-of-function mutation in the melanocytes and causes a pigmentary phenotype. Constitutional missense mutations at p.Arg1809 affect 1.23% of unrelated NF1 probands in the UAB cohort, therefore this specific NF1 genotype-phenotype correlation will affect counseling and management of a significant number of patients
Genetically engineered minipigs model the major clinical features of human neurofibromatosis type 1.
Neurofibromatosis Type 1 (NF1) is a genetic disease caused by mutations in Neurofibromin 1 (NF1). NF1 patients present with a variety of clinical manifestations and are predisposed to cancer development. Many NF1 animal models have been developed, yet none display the spectrum of disease seen in patients and the translational impact of these models has been limited. We describe a minipig model that exhibits clinical hallmarks of NF1, including café au lait macules, neurofibromas, and optic pathway glioma. Spontaneous loss of heterozygosity is observed in this model, a phenomenon also described in NF1 patients. Oral administration of a mitogen-activated protein kinase/extracellular signal-regulated kinase inhibitor suppresses Ras signaling. To our knowledge, this model provides an unprecedented opportunity to study the complex biology and natural history of NF1 and could prove indispensable for development of imaging methods, biomarkers, and evaluation of safety and efficacy of NF1-targeted therapies
Fungal microbiota from rain water and pathogenicity of Fusarium species isolated from atmospheric dust and rainfall dust
In order to determine the presence of Fusarium spp. in atmospheric dust and rainfall dust, samples were collected during September 2007, and July, August, and October 2008. The results reveal the prevalence of airborne Fusarium species coming from the atmosphere of the South East coast of Spain. Five different Fusarium species were isolated from the settling dust: Fusarium oxysporum, F. solani, F. equiseti, F. dimerum, and F. proliferatum. Moreover, rainwater samples were obtained during significant rainfall events in January and February 2009. Using the dilution-plate method, 12 fungal genera were identified from these rainwater samples. Specific analyses of the rainwater revealed the presence of three species of Fusarium: F. oxysporum, F. proliferatum and F. equiseti. A total of 57 isolates of Fusarium spp. obtained from both rainwater and atmospheric rainfall dust sampling were inoculated onto melon (Cucumis melo L.) cv. Piñonet and tomato (Lycopersicon esculentum Mill.) cv. San Pedro. These species were chosen because they are the main herbaceous crops in Almeria province. The results presented in this work indicate strongly that spores or propagules of Fusarium are able to cross the continental barrier carried by winds from the Sahara (Africa) to crop or coastal lands in Europe. Results show differences in the pathogenicity of the isolates tested. Both hosts showed root rot when inoculated with different species of Fusarium, although fresh weight measurements did not bring any information about the pathogenicity. The findings presented above are strong indications that long-distance transmission of Fusarium propagules may occur. Diseases caused by species of Fusarium are common in these areas. They were in the past, and are still today, a problem for greenhouses crops in AlmerÃa, and many species have been listed as pathogens on agricultural crops in this region. Saharan air masses dominate the Mediterranean regions. The evidence of long distance dispersal of Fusarium spp. by atmospheric dust and rainwater together with their proved pathogenicity must be taken into account in epidemiological studies
Typical 22q11.2 deletion syndrome appears to confer a reduced risk of schwannoma
Purpose: The LZTR1 gene has been associated with schwannomatosis tumor predisposition and is located in a region that is deleted in the great majority (89%) of patients with 22q11.2 deletion syndrome (22q11.2DS). Since it is known that approximately 1 in 500 people in the general population will develop a sporadic schwannoma and there are no reports of the occurrence of schwannoma in 22q11.2DS, we investigated whether whole-gene deletion of LZTR1 occurs in schwannomatosis and assessed the risk of schwannoma in 22q11.2DS. Methods: We assessed the genetic testing results for LZTR1-associated schwannomatosis and the clinical phenotypes of patients with 22q11.2DS. Results: There were no reports of schwannoma in over 1,500 patients with 22q11.2DS. In addition, no patients meeting clinical diagnostic criteria for schwannomatosis had a whole-gene deletion in LZTR1. Only 1 patient in 110 with an apparently sporadic vestibular schwannoma had a constitutional whole-gene deletion of LZTR1. Conclusion: People with a large 22q11.2 deletion may have a reduced risk of developing a schwannoma compared to the general population
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