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Sökning: WFRF:(Lerche E)

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1.
  • Murari, A., et al. (författare)
  • A control oriented strategy of disruption prediction to avoid the configuration collapse of tokamak reactors
  • 2024
  • Ingår i: Nature Communications. - 2041-1723 .- 2041-1723. ; 15:1
  • Tidskriftsartikel (refereegranskat)abstract
    • The objective of thermonuclear fusion consists of producing electricity from the coalescence of light nuclei in high temperature plasmas. The most promising route to fusion envisages the confinement of such plasmas with magnetic fields, whose most studied configuration is the tokamak. Disruptions are catastrophic collapses affecting all tokamak devices and one of the main potential showstoppers on the route to a commercial reactor. In this work we report how, deploying innovative analysis methods on thousands of JET experiments covering the isotopic compositions from hydrogen to full tritium and including the major D-T campaign, the nature of the various forms of collapse is investigated in all phases of the discharges. An original approach to proximity detection has been developed, which allows determining both the probability of and the time interval remaining before an incoming disruption, with adaptive, from scratch, real time compatible techniques. The results indicate that physics based prediction and control tools can be developed, to deploy realistic strategies of disruption avoidance and prevention, meeting the requirements of the next generation of devices.
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  • Krasilnikov, A., et al. (författare)
  • Evidence of 9 Be + p nuclear reactions during 2ω CH and hydrogen minority ICRH in JET-ILW hydrogen and deuterium plasmas
  • 2018
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 58:2
  • Tidskriftsartikel (refereegranskat)abstract
    • The intensity of 9Be + p nuclear fusion reactions was experimentally studied during second harmonic (2ω CH) ion-cyclotron resonance heating (ICRH) and further analyzed during fundamental hydrogen minority ICRH of JET-ILW hydrogen and deuterium plasmas. In relatively low-density plasmas with a high ICRH power, a population of fast H+ ions was created and measured by neutral particle analyzers. Primary and secondary nuclear reaction products, due to 9Be + p interaction, were observed with fast ion loss detectors, γ-ray spectrometers and neutron flux monitors and spectrometers. The possibility of using 9Be(p, d)2α and 9Be(p, α)6Li nuclear reactions to create a population of fast alpha particles and study their behaviour in non-active stage of ITER operation is discussed in the paper.
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  • 2018
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 58:1
  • Forskningsöversikt (refereegranskat)
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  • Bombarda, F., et al. (författare)
  • Runaway electron beam control
  • 2019
  • Ingår i: Plasma Physics and Controlled Fusion. - : IOP Publishing. - 1361-6587 .- 0741-3335. ; 61:1
  • Tidskriftsartikel (refereegranskat)
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13.
  • Joffrin, E., et al. (författare)
  • Overview of the JET preparation for deuterium-tritium operation with the ITER like-wall
  • 2019
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 59:11
  • Forskningsöversikt (refereegranskat)abstract
    • For the past several years, the JET scientific programme (Pamela et al 2007 Fusion Eng. Des. 82 590) has been engaged in a multi-campaign effort, including experiments in D, H and T, leading up to 2020 and the first experiments with 50%/50% D-T mixtures since 1997 and the first ever D-T plasmas with the ITER mix of plasma-facing component materials. For this purpose, a concerted physics and technology programme was launched with a view to prepare the D-T campaign (DTE2). This paper addresses the key elements developed by the JET programme directly contributing to the D-T preparation. This intense preparation includes the review of the physics basis for the D-T operational scenarios, including the fusion power predictions through first principle and integrated modelling, and the impact of isotopes in the operation and physics of D-T plasmas (thermal and particle transport, high confinement mode (H-mode) access, Be and W erosion, fuel recovery, etc). This effort also requires improving several aspects of plasma operation for DTE2, such as real time control schemes, heat load control, disruption avoidance and a mitigation system (including the installation of a new shattered pellet injector), novel ion cyclotron resonance heating schemes (such as the three-ions scheme), new diagnostics (neutron camera and spectrometer, active Alfven eigenmode antennas, neutral gauges, radiation hard imaging systems...) and the calibration of the JET neutron diagnostics at 14 MeV for accurate fusion power measurement. The active preparation of JET for the 2020 D-T campaign provides an incomparable source of information and a basis for the future D-T operation of ITER, and it is also foreseen that a large number of key physics issues will be addressed in support of burning plasmas.
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  • Overview of the JET results
  • 2015
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 0029-5515 .- 1741-4326. ; 55:10
  • Tidskriftsartikel (refereegranskat)
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  • 2018
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 58:9
  • Tidskriftsartikel (refereegranskat)
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31.
  • Romanelli, F, et al. (författare)
  • Overview of the JET results
  • 2011
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 51:9
  • Tidskriftsartikel (refereegranskat)abstract
    • Since the last IAEA Conference JET has been in operation for one year with a programmatic focus on the qualification of ITER operating scenarios, the consolidation of ITER design choices and preparation for plasma operation with the ITER-like wall presently being installed in JET. Good progress has been achieved, including stationary ELMy H-mode operation at 4.5 MA. The high confinement hybrid scenario has been extended to high triangularity, lower ρ*and to pulse lengths comparable to the resistive time. The steady-state scenario has also been extended to lower ρ*and ν*and optimized to simultaneously achieve, under stationary conditions, ITER-like values of all other relevant normalized parameters. A dedicated helium campaign has allowed key aspects of plasma control and H-mode operation for the ITER non-activated phase to be evaluated. Effective sawtooth control by fast ions has been demonstrated with3He minority ICRH, a scenario with negligible minority current drive. Edge localized mode (ELM) control studies using external n = 1 and n = 2 perturbation fields have found a resonance effect in ELM frequency for specific q95values. Complete ELM suppression has, however, not been observed, even with an edge Chirikov parameter larger than 1. Pellet ELM pacing has been demonstrated and the minimum pellet size needed to trigger an ELM has been estimated. For both natural and mitigated ELMs a broadening of the divertor ELM-wetted area with increasing ELM size has been found. In disruption studies with massive gas injection up to 50% of the thermal energy could be radiated before, and 20% during, the thermal quench. Halo currents could be reduced by 60% and, using argon/deuterium and neon/deuterium gas mixtures, runaway electron generation could be avoided. Most objectives of the ITER-like ICRH antenna have been demonstrated; matching with closely packed straps, ELM resilience, scattering matrix arc detection and operation at high power density (6.2 MW m-2) and antenna strap voltages (42 kV). Coupling measurements are in very good agreement with TOPICA modelling. © 2011 IAEA, Vienna.
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32.
  • Abel, I, et al. (författare)
  • Overview of the JET results with the ITER-like wall
  • 2013
  • Ingår i: Nuclear Fusion. - : IOP Publishing. - 1741-4326 .- 0029-5515. ; 53:10, s. 104002-
  • Tidskriftsartikel (refereegranskat)abstract
    • Following the completion in May 2011 of the shutdown for the installation of the beryllium wall and the tungsten divertor, the first set of JET campaigns have addressed the investigation of the retention properties and the development of operational scenarios with the new plasma-facing materials. The large reduction in the carbon content (more than a factor ten) led to a much lower Z(eff) (1.2-1.4) during L- and H-mode plasmas, and radiation during the burn-through phase of the plasma initiation with the consequence that breakdown failures are almost absent. Gas balance experiments have shown that the fuel retention rate with the new wall is substantially reduced with respect to the C wall. The re-establishment of the baseline H-mode and hybrid scenarios compatible with the new wall has required an optimization of the control of metallic impurity sources and heat loads. Stable type-I ELMy H-mode regimes with H-98,H-y2 close to 1 and beta(N) similar to 1.6 have been achieved using gas injection. ELM frequency is a key factor for the control of the metallic impurity accumulation. Pedestal temperatures tend to be lower with the new wall, leading to reduced confinement, but nitrogen seeding restores high pedestal temperatures and confinement. Compared with the carbon wall, major disruptions with the new wall show a lower radiated power and a slower current quench. The higher heat loads on Be wall plasma-facing components due to lower radiation made the routine use of massive gas injection for disruption mitigation essential.
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  • Hobirk, J., et al. (författare)
  • The JET hybrid scenario in Deuterium, Tritium and Deuterium-Tritium
  • 2023
  • Ingår i: Nuclear Fusion. - : Institute of Physics Publishing (IOPP). - 0029-5515 .- 1741-4326. ; 63:11
  • Tidskriftsartikel (refereegranskat)abstract
    • The JET hybrid scenario has been developed from low plasma current carbon wall discharges to the record-breaking Deuterium-Tritium plasmas obtained in 2021 with the ITER-like Be/W wall. The development started in pure Deuterium with refinement of the plasma current, and toroidal magnetic field choices and succeeded in solving the heat load challenges arising from 37 MW of injected power in the ITER like wall environment, keeping the radiation in the edge and core controlled, avoiding MHD instabilities and reaching high neutron rates. The Deuterium hybrid plasmas have been re-run in Tritium and methods have been found to keep the radiation controlled but not at high fusion performance probably due to time constraints. For the first time this scenario has been run in Deuterium-Tritium (50:50). These plasmas were re-optimised to have a radiation-stable H-mode entry phase, good impurity control through edge Ti gradient screening and optimised performance with fusion power exceeding 10 MW for longer than three alpha particle slow down times, 8.3 MW averaged over 5 s and fusion energy of 45.8 MJ.
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  • Helbig, K. L., et al. (författare)
  • De Novo Pathogenic Variants in CACNA1E Cause Developmental and Epileptic Encephalopathy with Contractures, Macrocephaly, and Dyskinesias
  • 2018
  • Ingår i: American Journal of Human Genetics. - : Elsevier BV. - 0002-9297 .- 1537-6605. ; 103:5, s. 666-678
  • Tidskriftsartikel (refereegranskat)abstract
    • Developmental and epileptic encephalopathies (DEEs) are severe neurodevelopmental disorders often beginning in infancy or early childhood that are characterized by intractable seizures, abundant epileptiform activity on EEG, and developmental impairment or regression. CACNA1E is highly expressed in the central nervous system and encodes the alpha(1)-subunit of the voltage-gated Ca(V)2.3 channel, which conducts high voltage-activated R-type calcium currents that initiate synaptic transmission. Using next-generation sequencing techniques, we identified de novo CACNA1E variants in 30 individuals with DEE, characterized by refractory infantile-onset seizures, severe hypotonia, and profound developmental impairment, often with congenital contractures, macrocephaly, hyperkinetic movement disorders, and early death. Most of the 14, partially recurring, variants cluster within the cytoplasmic ends of all four S6 segments, which form the presumed Ca(V)2.3 channel activation gate. Functional analysis of several S6 variants revealed consistent gain-of-function effects comprising facilitated voltage-dependent activation and slowed inactivation. Another variant located in the domain II S4-S5 linker results in facilitated activation and increased current density. Five participants achieved seizure freedom on the anti-epileptic drug topiramate, which blocks R-type calcium channels. We establish pathogenic variants in CACNA1E as a cause of DEEs and suggest facilitated R-type calcium currents as a disease mechanism for human epilepsy and developmental disorders.
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  • Kiptily, V. G., et al. (författare)
  • Observation of alpha-particles in recent D-T experiments on JET
  • 2024
  • Ingår i: Nuclear Fusion. - : Institute of Physics (IOP). - 0029-5515 .- 1741-4326. ; 64:8
  • Tidskriftsartikel (refereegranskat)abstract
    • The fusion reaction between deuterium and tritium, D(T,n)4 He is the main source of energy in future thermonuclear reactors. Alpha-particles (4 He-ions) born with an average energy of 3.5 MeV transferring energy to the thermal plasma during their slowing down, should provide the self-sustained D-T plasma burn. The adequate confinement of alpha-particles is essential to provide efficient heating of the bulk plasma and steady burning of a reactor plasma. That is why the fusion-born alpha-particle studies have been a priority task in the second D-T experiments (DTE2) on the Joint European Torus (JET) to understand the main mechanisms of their slowing down, redistribution and losses and to develop optimal plasma scenarios. JET with Be-wall and W-divertor, enhanced auxiliary heating systems and improved energetic-particle diagnostic capabilities, producing significant population of alpha-particles, provided the possibility for comprehensive studying of the alpha-particle behaviour. Selected results of the confined and lost alpha-particle measurements, evidence of alpha-particle self-heating and assessments of the fusion performance are presented in this paper giving an opportunity for further modelling and extrapolation to the International Thermonuclear Experimental Reactor and burning plasma reactors.
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37.
  • Nocente, M., et al. (författare)
  • Generation and observation of fast deuterium ions and fusion-born alpha particles in JET D-He-3 plasmas with the 3-ion radio-frequency heating scenario
  • 2020
  • Ingår i: Nuclear Fusion. - : IOP PUBLISHING LTD. - 0029-5515 .- 1741-4326. ; 60:12
  • Tidskriftsartikel (refereegranskat)abstract
    • Dedicated experiments to generate energetic D ions and D-(3) He fusion-born alpha particles were performed at the Joint European Torus (JET) with the ITER-like wall (ILW). Using the 3-ion D-(D-NBI)-(3) He radio frequency (RF) heating scenario, deuterium ions from neutral beam injection (NBI) were accelerated in the core of mixed D-(3) He plasmas to higher energies with ion cyclotron resonance frequency (ICRF) waves, in turn leading to a core-localized source of alpha particles. The fast-ion distribution of RF-accelerated D-NBI ions was controlled by varying the ICRF and NBI power (P-ICRF approximate to 4-6 MW, P-NBI approximate to 3-20 MW), resulting in rather high D-D neutron (approximate to 1x10(16) s(-1)) and D-(3) He alpha rates (approximate to 2x10(16) s(-1)) at moderate input heating power. Theory and TRANSP analysis shows that large populations of co-passing MeV-range D ions were generated using the D-(D-NBI)-(3) He 3-ion ICRF scenario. This important result is corroborated by several experimental observations, in particular gamma-ray measurements. The developed experimental scenario at JET provides unique conditions for probing several aspects of future burning plasmas, such as the contribution from MeV range ions to global confinement, but without introducing tritium. Dominant fast-ion core electron heating with T-i approximate to T-e and a rich variety of fast-ion driven Alfven eigenmodes (AEs) were observed in these D-(3) He plasmas. The observed AE activities do not have a detrimental effect on the thermal confinement and, in some cases, may be driven by the fusion born alpha particles. A strong continuous increase in neutron rate was observed during long-period sawteeth (>1 s), accompanied by the observation of reversed shear AEs, which implies that a non monotonic q profile was systematically developed in these plasmas, sustained by the large fast-ion populations generated by the 3-ion ICRF scenario.
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  • Mantica, P., et al. (författare)
  • Detection of alpha heating in JET-ILW DT plasmas by a study of the electron temperature response to ICRH modulation
  • 2024
  • Ingår i: Nuclear Fusion. - Culham Sci Ctr, JET, EUROfus Consortium, Abingdon OX14 3DB, England. [Mantica, P.; Casiraghi, I.; Dal Molin, A.; Rigamonti, D.; Tardocchi, M.] CNR, Inst Plasma Sci & Technol, Milan, Italy. [Auriemma, F.] Consorzio RFX ISTP CNR, Padua, Italy. [Gallart, D.; Mantsinen, M.] Barcelona Supercomp Ctr, Barcelona, Spain. [Kirov, K.; Jacquet, P.; Kiptily, V.; Litherland-Smith, E.; Maggi, C. F.; Maslov, M.; Menmuir, S.; Stancar, Z.; Sun, H.; Szepesi, G.] Culham Sci Ctr, UKAEA, Abingdon, England. [Lerche, E.; Van Eester, D.] ERM KMS, Lab Plasma Phys, Brussels, Belgium. [Salmi, A.] VTT, POB 1000, FI-02044 Espoo, Finland. [Delabie, E.] Oak Ridge Natl Lab, Oak Ridge, TN 37831 USA. [Eriksson, J.] Uppsala Univ, Dept Phys & Astron, Uppsala, Sweden. [Garcia, J.; Huynh, P.] CEA, IRFM, St Paul Les Durance, France. [$$$Jonsson, T.] KTH, Fus Plasma Phys, EES, Stockholm, Sweden. [Mantsinen, M.] ICREA, Barcelona, Spain. [Marcer, G.; Nocente, M.] Univ Milano Bicocca, Milan, Italy. [Peluso, E.] Univ Roma Tor Vergata, Rome, Italy. [Peluso, E.; Pucella, G.] ENEA CR Frascati, Frascati, Italy. : IOP Publishing. - 0029-5515 .- 1741-4326. ; 64:8
  • Tidskriftsartikel (refereegranskat)abstract
    • In the JET DTE2 campaign a new method was successfully tested to detect the heating of bulk electrons by alpha-particles, using the dynamic response of the electron temperature T e to the modulation of ion cyclotron resonance heating (ICRH). A fundamental deuterium (D) ICRH scheme was applied to a tritium-rich hybrid plasma with D-neutral beam injection (NBI). The modulation of the ion temperature T i and of the ICRH accelerated deuterons leads to modulated alpha-heating with a large delay with respect to other modulated electron heating terms. A significant phase delay of similar to 40 degrees is measured between central T e and T i, which can only be explained by alpha-particle heating. Integrated modelling using different models for ICRH absorption and ICRH/NBI interaction reproduces the effect qualitatively. Best agreement with experiment is obtained with the European Transport Solver/Heating and Current Drive workflow.
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  • Mantsinen, M. J., et al. (författare)
  • Experiments in high-performance JET plasmas in preparation of second harmonic ICRF heating of tritium in ITER
  • 2023
  • Ingår i: Nuclear Fusion. - : Institute of Physics (IOP). - 0029-5515 .- 1741-4326. ; 63:11
  • Tidskriftsartikel (refereegranskat)abstract
    • The reference ion cyclotron resonance frequency (ICRF) heating schemes for ITER deuterium-tritium (D-T) plasmas at the full magnetic field of 5.3 T are second harmonic heating of T and 3He minority heating. The wave-particle resonance location for these schemes coincide and are central at a wave frequency of 53 MHz at 5.3 T. Experiments have been carried out in the second major D-T campaign (DTE2) at JET, and in its prior D campaigns, to integrate these ICRF scenarios in JET high-performance plasmas and to compare their performance with the commonly used hydrogen (H) minority heating. In 50:50 D:T plasmas, up to 35% and 5% larger fusion power and diamagnetic energy content, respectively, were obtained with second harmonic heating of T as compared to H minority heating at comparable total input powers and gas injection rates. The core ion temperature was up to 30% and 20% higher with second harmonic T and 3He minority heating, respectively, with respect to H minority heating. These are favourable results for the use of these scenarios in ITER and future fusion reactors. According to modelling, adding ICRF heating to neutral beam injection using D and T beams resulted in a 10%-20% increase of on-axis bulk ion heating in the D-T plasmas due to its localisation in the plasma core. Central power deposition was confirmed with the break-in-slope and fast Fourier transform analysis of ion and electron temperature in response to ICRF modulation. The tail temperature of fast ICRF-accelerated tritons, their enhancement of the fusion yield and time behaviour as measured by an upgraded magnetic proton recoil spectrometer and neutral particle analyser were found in agreement with theoretical predictions. No losses of ICRF-accelerated ions were observed by fast ion detectors, which was as expected given the high plasma density of n e approximate to 7-8 x 1019 m-3 in the main heating phase that limited the formation of ICRF-accelerated fast ion tails. 3He was introduced in the machine by 3He gas injection, and the 3He concentration was measured by a high-resolution optical penning gauge in the sub-divertor region. The DTE2 experiments with 3He minority heating were carried with a low 3He concentration in the range of 2%-4% given the fact that the highest neutron rates with 3He minority heating in D plasmas were obtained at low 3He concentrations of similar to 2%, which also coincided with the highest plasma diamagnetic energy content. In addition to 3He introduced by 3He gas injection, an intrinsic concentration of 3He of the order of 0.2%-0.4% was measured in D-T plasmas before 3He was introduced in the device, which is attributed to the radioactive decay of tritium to 3He. According to modelling, even such low intrinsic concentrations of 3He lead to significant changes in ICRF power partitioning during second harmonic heating of T due to absorption of up to 30% of the wave power by 3He.
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  • Maslov, M., et al. (författare)
  • JET D-T scenario with optimized non-thermal fusion
  • 2023
  • Ingår i: Nuclear Fusion. - : Institute of Physics (IOP). - 0029-5515 .- 1741-4326. ; 63:11
  • Tidskriftsartikel (refereegranskat)abstract
    • In JET deuterium-tritium (D-T) plasmas, the fusion power is produced through thermonuclear reactions and reactions between thermal ions and fast particles generated by neutral beam injection (NBI) heating or accelerated by electromagnetic wave heating in the ion cyclotron range of frequencies (ICRFs). To complement the experiments with 50/50 D/T mixtures maximizing thermonuclear reactivity, a scenario with dominant non-thermal reactivity has been developed and successfully demonstrated during the second JET deuterium-tritium campaign DTE2, as it was predicted to generate the highest fusion power in JET with a Be/W wall. It was performed in a 15/85 D/T mixture with pure D-NBI heating combined with ICRF heating at the fundamental deuterium resonance. In steady plasma conditions, a record 59 MJ of fusion energy has been achieved in a single pulse, of which 50.5 MJ were produced in a 5 s time window (P fus = 10.1 MW) with average Q = 0.33, confirming predictive modelling in preparation of the experiment. The highest fusion power in these experiments, P fus = 12.5 MW with average Q = 0.38, was achieved over a shorter 2 s time window, with the period of sustainment limited by high-Z impurity accumulation. This scenario provides unique data for the validation of physics-based models used to predict D-T fusion power.
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  • Van Eester, D, et al. (författare)
  • JET (He-3)-D scenarios relying on RF heating : survey of selected recent experiments
  • 2009
  • Ingår i: Plasma Physics and Controlled Fusion. - : IOP Publishing. - 0741-3335 .- 1361-6587. ; 51:4, s. 044007-
  • Tidskriftsartikel (refereegranskat)abstract
    • Recent JET experiments have been devoted to the study of (He-3)-D plasmas involving radio frequency (RF) heating. This paper starts by discussing the RF heating efficiency theoretically expected in such plasmas, covering both relevant aspects of wave and of particle dynamics. Then it gives a concise summary of the main conclusions drawn from recent experiments that were either focusing on studying RF heating physics aspects or that were adopting RF heating as a tool to study plasma behavior. Depending on the minority concentration chosen, different physical phenomena are observed. At very low concentration (X[He-3] < 1%), energetic tails are formed which trigger MHD activity and result in loss of fast particles. Alfven cascades were observed and gamma ray tomography indirectly shows the impact of sawtooth crashes on the fast particle orbits. Low concentration (X[He-3] < 10%) favors minority heating while for X[He-3] >> 10% electron mode conversion damping becomes dominant. Evidence for the Fuchs et al standing wave effect (Fuchs et al 1995 Phys. Plasmas 2 1637-47) on the absorption is presented. RF induced deuterium tails were observed in mode conversion experiments with large X[He-3] (approximate to 18%). As tentative modeling shows, the formation of these tails can be explained as a consequence of wave power absorption by neutral beam particles that efficiently interact with the waves well away from the cold D cyclotron resonance position as a result of their substantial Doppler shift. As both ion and electron RF power deposition profiles in (He-3)-D plasmas are fairly narrow-giving rise to localized heat sources-the RF heating method is an ideal tool for performing transport studies. Various of the experiments discussed here were done in plasmas with internal transport barriers (ITBs). ITBs are identified as regions with locally reduced diffusivity, where poloidal spinning up of the plasma is observed. The present know-how on the role of RF heating for impurity transport is also briefly summarized.
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45.
  • Chatron, N., et al. (författare)
  • Bi-allelic GAD1 variants cause a neonatal onset syndromic developmental and epileptic encephalopathy
  • 2020
  • Ingår i: Brain. - : Oxford University Press (OUP). - 0006-8950 .- 1460-2156. ; 143:5, s. 1447-1461
  • Tidskriftsartikel (refereegranskat)abstract
    • Developmental and epileptic encephalopathies are a heterogeneous group of early-onset epilepsy syndromes dramatically impairing neurodevelopment. Modern genomic technologies have revealed a number of monogenic origins and opened the door to therapeutic hopes. Here we describe a new syndromic developmental and epileptic encephalopathy caused by bi-allelic loss-of-function variants in GAD1, as presented by 11 patients from six independent consanguineous families. Seizure onset occurred in the first 2 months of life in all patients. All 10 patients, from whom early disease history was available, presented with seizure onset in the first month of life, mainly consisting of epileptic spasms or myoclonic seizures. Early EEG showed suppression-burst or pattern of burst attenuation or hypsarrhythmia if only recorded in the post-neonatal period. Eight patients had joint contractures and/or pes equinovarus. Seven patients presented a cleft palate and two also had an omphalocele, reproducing the phenotype of the knockout Gad1(-/-) mouse model. Four patients died before 4 years of age. GAD1 encodes the glutamate decarboxylase enzyme GAD67, a critical actor of the c-aminobutyric acid (GABA) metabolism as it catalyses the decarboxylation of glutamic acid to form GABA. Our findings evoke a novel syndrome related to GAD67 deficiency, characterized by the unique association of developmental and epileptic encephalopathies, cleft palate, joint contractures and/or omphalocele.
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49.
  • Kim, Hyun-Tae, et al. (författare)
  • Validation of D-T fusion power prediction capability against 2021 JET D-T experiments
  • 2023
  • Ingår i: Nuclear Fusion. - 0029-5515 .- 1741-4326. ; 63:11
  • Tidskriftsartikel (refereegranskat)abstract
    • JET experiments using the fuel mixture envisaged for fusion power plants, deuterium and tritium (D-T), provide a unique opportunity to validate existing D-T fusion power prediction capabilities in support of future device design and operation preparation. The 2021 JET D-T experimental campaign has achieved D-T fusion powers sustained over 5 s in ITER-relevant conditions i.e. operation with the baseline or hybrid scenario in the full metallic wall. In preparation of the 2021 JET D-T experimental campaign, extensive D-T predictive modelling was carried out with several assumptions based on D discharges. To improve the validity of ITER D-T predictive modelling in the future, it is important to use the input data measured from 2021 JET D-T discharges in the present core predictive modelling, and to specify the accuracy of the D-T fusion power prediction in comparison with the experiments. This paper reports on the validation of the core integrated modelling with TRANSP, JINTRAC, and ETS coupled with a quasilinear turbulent transport model (Trapped Gyro Landau Fluid or QualLiKiz) against the measured data in 2021 JET D-T discharges. Detailed simulation settings and the heating and transport models used are described. The D-T fusion power calculated with the interpretive TRANSP runs for 38 D-T discharges (12 baseline and 26 hybrid discharges) reproduced the measured values within 20 % . This indicates the additional uncertainties, that could result from the measurement error bars in kinetic profiles, impurity contents and neutron rates, and also from the beam-thermal fusion reaction modelling, are less than 20 % in total. The good statistical agreement confirms that we have the capability to accurately calculate the D-T fusion power if correct kinetic profiles are predicted, and indicates that any larger deviation of the D-T fusion power prediction from the measured fusion power could be attributed to the deviation of the predicted kinetic profiles from the measured kinetic profiles in these plasma scenarios. Without any posterior adjustment of the simulation settings, the ratio of predicted D-T fusion power to the measured fusion power was found as 65%-96% for the D-T baseline and 81%-97% for D-T hybrid discharge. Possible reasons for the lower D-T prediction are discussed and future works to improve the fusion power prediction capability are suggested. The D-T predictive modelling results have also been compared to the predictive modelling of the counterpart D discharges, where the key engineering parameters are similar. Features in the predicted kinetic profiles of D-T discharges such as underprediction of ne are also found in the prediction results of the counterpart D discharges, and it leads to similar levels of the normalized neutron rate prediction between the modelling results of D-T and the counterpart D discharges. This implies that the credibility of D-T fusion power prediction could be a priori estimated by the prediction quality of the preparatory D discharges, which will be attempted before actual D-T experiments.
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50.
  • Lennholm, M., et al. (författare)
  • Real-time control of ELM and sawtooth frequencies : Similarities and differences
  • 2015
  • Ingår i: Nuclear Fusion. - : Institute of Physics Publishing (IOPP). - 0029-5515 .- 1741-4326. ; 56:1
  • Tidskriftsartikel (refereegranskat)abstract
    • ELMs and Sawteeth, located in different parts of the plasma, are similar from a control engineering point of view. Both manifest themselves through quiescent periods interrupted by periodic collapses. For both, large collapses, following long quiescent periods, have detrimental effects while short periods are associated with decreased confinement. Following the installation of the all metal ’ITER like wall’ on JET, sawteeth and ELMs also play an important role by expelling tungsten from the core and edge of the plasma respectively. Control of tungsten has therefore been added to divertor heat load reduction, NTM avoidance and helium ash removal as reasons for requiring ELM and sawtooth control. It is therefore of interest to implement control systems to maintain the sawtooth and ELM frequencies in the desired ranges. On JET, ELM frequency control uses radial field ’kicks’ and pellet and gas injection as actuators, while sawtooth control uses ion cyclotron resonance heating (ICRH). JET experiments have, for the first time, established feedback control of the ELM frequency, via real time variation of the injected gas flow [1]. Using this controller in conjunction with pellet injection allows the ELM frequency to be kept as required despite variations in pellet ELM triggering efficiency. JET Sawtooth control experiments have, for the first time, demonstrated that low field side ICRH, as foreseen for ITER, can shorten sawteeth lengthened by central fast ions [2]. The development of ELM and sawtooth control could be key to achieve stable high performance JET discharges with minimal tungsten content. Integrating such schemes into an overall control strategy will be required in future tokamaks and gaining experience on current tokamaks is essential.
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