114 research outputs found

    Beam-based characterization of plasma density in a capillary-discharge waveguide

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    Next-generation plasma-based accelerators can push electron bunches to gigaelectronvolt energies within centimeter distances. In these devices, the accelerating force is provided by a driver pulse, either a laser pulse or a particle bunch, that loses its energy into the plasma generating huge electric fields up to tens of GV/m. The stability of such fields strongly depends on plasma density, whose exact value should be precisely known and controlled. However, currently available methods based on spectroscopic or interferometric techniques find it very difficult to measure plasma density lower than 1015–16 cm−3 in capillary-discharge waveguides. Here, we present a novel diagnostic tool that allows us to estimate the average density of a plasma capillary by probing it with an ultra-relativistic electron beam. The plasma density and the generated accelerating field are inferred by analyzing the beam longitudinal phase space after its interaction with the plasma. The results are validated by simulations showing excellent agreement

    Calcium mishandling in absence of primary mitochondrial dysfunction drives cellular pathology in Wolfram Syndrome

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    Wolfram syndrome (WS) is a recessive multisystem disorder defined by the association of diabetes mellitus and optic atrophy, reminiscent of mitochondrial diseases. The role played by mitochondria remains elusive, with contradictory results on the occurrence of mitochondrial dysfunction. We evaluated 13 recessive WS patients by deep clinical phenotyping, including optical coherence tomography (OCT), serum lactic acid at rest and after standardized exercise, brain Magnetic Resonance Imaging, and brain and muscle Magnetic Resonance Spectroscopy (MRS). Finally, we investigated mitochondrial bioenergetics, network morphology, and calcium handling in patient-derived fibroblasts. Our results do not support a primary mitochondrial dysfunction in WS patients, as suggested by MRS studies, OCT pattern of retinal nerve fiber layer loss, and, in fibroblasts, by mitochondrial bioenergetics and network morphology results. However, we clearly found calcium mishandling between endoplasmic reticulum (ER) and mitochondria, which, under specific metabolic conditions of increased energy requirements and in selected tissue or cell types, may turn into a secondary mitochondrial dysfunction. Critically, we showed that Wolframin (WFS1) protein is enriched at mitochondrial-associated ER membranes and that in patient-derived fibroblasts WFS1 protein is completely absent. These findings support a loss-of-function pathogenic mechanism for missense mutations in WFS1, ultimately leading to defective calcium influx within mitochondria

    First emittance measurement of the beam-driven plasma wakefield accelerated electron beam

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    Next-generation plasma-based accelerators can push electron beams to GeV energies within centimetre distances. The plasma, excited by a driver pulse, is indeed able to sustain huge electric fields that can efficiently accelerate a trailing witness bunch, which was experimentally demonstrated on multiple occasions. Thus, the main focus of the current research is being shifted towards achieving a high quality of the beam after the plasma acceleration. In this letter we present beam-driven plasma wakefield acceleration experiment, where initially preformed high-quality witness beam was accelerated inside the plasma and characterized. In this experiment the witness beam quality after the acceleration was maintained on high level, with 0.2%0.2\% final energy spread and 3.8 μm3.8~\mu m resulting normalized transverse emittance after the acceleration. In this article, for the first time to our knowledge, the emittance of the PWFA beam was directly measured

    First emittance measurement of the beam-driven plasma wakefield accelerated electron beam

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    Next-generation plasma-based accelerators can push electron beams to GeV energies within centimeter distances. The plasma, excited by a driver pulse, is indeed able to sustain huge electric fields that can efficiently accelerate a trailing witness bunch, which was experimentally demonstrated on multiple occasions. Thus, the main focus of the current research is being shifted towards achieving a high quality of the beam after the plasma acceleration. In this paper we present a beam-driven plasma wakefield acceleration experiment, where initially preformed high-quality witness beam was accelerated inside the plasma and characterized. In this experiment the witness beam quality after the acceleration was maintained on high level, with 0.2% final energy spread and 3.8 μm resulting normalized transverse emittance after the acceleration. In this article, for the first time to our knowledge, the emittance of the plasma wakefield accelerated beam was directly measure

    ATPase Domain AFG3L2 Mutations Alter OPA1 Processing and Cause Optic Neuropathy

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    Objective: Dominant optic atrophy (DOA) is the most common inherited optic neuropathy, with a prevalence of 1:12,000 to 1:25,000. OPA1 mutations are found in 70% of DOA patients, with a significant number remaining undiagnosed. Methods: We screened 286 index cases presenting optic atrophy, negative for OPA1 mutations, by targeted next generation sequencing or whole exome sequencing. Pathogenicity and molecular mechanisms of the identified variants were studied in yeast and patient-derived fibroblasts. Results: Twelve cases (4%) were found to carry novel variants in AFG3L2, a gene that has been associated with autosomal dominant spinocerebellar ataxia 28 (SCA28). Half of cases were familial with a dominant inheritance, whereas the others were sporadic, including de novo mutations. Biallelic mutations were found in 3 probands with severe syndromic optic neuropathy, acting as recessive or phenotype-modifier variants. All the DOA-associated AFG3L2 mutations were clustered in the ATPase domain, whereas SCA28-associated mutations mostly affect the proteolytic domain. The pathogenic role of DOA-associated AFG3L2 mutations was confirmed in yeast, unraveling a mechanism distinct from that of SCA28-associated AFG3L2 mutations. Patients' fibroblasts showed abnormal OPA1 processing, with accumulation of the fission-inducing short forms leading to mitochondrial network fragmentation, not observed in SCA28 patients' cells. Interpretation: This study demonstrates that mutations in AFG3L2 are a relevant cause of optic neuropathy, broadening the spectrum of clinical manifestations and genetic mechanisms associated with AFG3L2 mutations, and underscores the pivotal role of OPA1 and its processing in the pathogenesis of DOA. ANN NEUROL 2020

    Energy spread minimization in a beam-driven plasma wakefield accelerator

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    Next-generation plasma-based accelerators can push electron bunches to gigaelectronvolt energies within centimetre distances. The plasma, excited by a driver pulse, generates large electric fields that can efficiently accelerate a trailing witness bunch making possible the realization of laboratory-scale applications ranging from high-energy colliders to ultra-bright light sources. So far several experiments have demonstrated a significant acceleration but the resulting beam quality, especially the energy spread, is still far from state of the art conventional accelerators. Here we show the results of a beam-driven plasma acceleration experiment where we used an electron bunch as a driver followed by an ultra-short witness. The experiment demonstrates, for the first time, an innovative method to achieve an ultra-low energy spread of the accelerated witness of about 0.1%. This is an order of magnitude smaller than what has been obtained so far. The result can lead to a major breakthrough toward the optimization of the plasma acceleration process and its implementation in forthcoming compact machines for user-oriented applications

    Melhoramento genético de trigo via linhagens duplo-haplóides, na Embrapa Trigo, no período 1995-1998.

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    Visando a aceleração no processo de obtenção de linhagens de trigo e a melhoria das condições de seleção, em material geneticamente fixo (homozigoto), foi implementada a produção de novas linhagens duplo-haplóides (DH), via gimnogenese (DHM), no projeto melhoramento genético de trigo para o Brasil, conduzido na Embrapa Trigo, em Passo Fundo, Rio Grande do Sul. Com a utilização dessa metodologia, que permite a criação de linhagens geneticamente uniformes, em menos de dois anos, a partir do cruzamento trigo X milho, o tempo para a criação de novas linhagens e cultivares foi reduzido de dois a quatro anos, considerando para comparação, respectivamente, o método convencional e o método rápido, que utiliza o avanço de geração no verão. No período 1995-1998 foram polinizadas, com milho, 6.988 espigas de trigo, resultando em 12.178 embriões resgatados e em 4.003 linhagens duplo-haplóides. Em 1998, havia uma coleção de, aproximadamente, 675 linhagens em multiplicação no campo, tendo sido selecionadas 262, que passaram para experimentação preliminar (1. ano / 1 local), em 1999, em ensaios com três repetições, além dessas, foram selecionadas 5 das 39 linhagens duplo-haplóides que estavam no ensaio preliminar em rede (2. ano / 6 locais), em 1999. Paralelamente, as linhagens foram avaliadas para resistência as principais doenças e, também, quanto a aptidão de uso industrial, empregando-se a eletroforese de gluteninas de alto peso molecular. Das 267 (262+5) linhagens selecionadas por características agronômicas, para inclusão e/ou permanência em ensaios de rendimento, em 1999, 51 (19,1%) e 42 (15,7%) apresentaram, respectivamente, combinação de 3 e de 2 bandas indicativas de boa aptidão panificativa

    Eupraxia, a step toward a plasma-wakefield based accelerator with high beam quality

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    The EuPRAXIA project aims at designing the world's first accelerator based on advanced plasma-wakefield techniques to deliver 5 GeV electron beams that simultaneously have high charge, low emittance and low energy spread, which are required for applications by future user communities. Meeting this challenging objective will only be possible through dedicated effort. Many injection/acceleration schemes and techniques have been explored by means of thorough simulations in more than ten European research institutes. This enables selection of the most appropriate methods for solving each particular problem. The specific challenge of generating, extracting and transporting high charge beams, while maintaining the high quality needed for user applications, are being tackled using innovative approaches. This article highlights preliminary results obtained by the EuPRAXIA collaboration, which also exhibit the required laser and plasma parameters
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