NIO1 experiment, a source of negative hydrogen and deuterium ions accelerated up to 60kV: commissioning and analysis of first experimental results of ion beam extraction

La tesi tratta della preparazione e della messa in servizio dell'esperimento NIO1, un acceleratore di ioni negativi d'idrogeno e deuterio e l'analisi dei primi risultati sperimentali. Saranno descritte le principali diagnostiche sviluppate ed installate su NIO1 e le eventuali problematiche connesse con questo esperimentoope

A strategy to identify breakdown location in MITICA test facility: results of high voltage test campaign

The Acceleration Grid Power Supply of the MITICA test facility in Padova (Italy) is currently under commissioning. The power conversion system, the DC generator, and the High Voltage equipment have been individually commissioned, whereas the integration tests are ongoing. It is a challenging process due to the unconventional application, to the variety of different electrical technologies involved and to the complexity of the interfaces. During the integrated tests of the power supplies the achievement of 700kV stable operation has been demonstrated for the first time in a Neutral Beam Injector, but an unexpected event occurred, most likely a breakdown in the HV part, which resulted in a fault of the DC generator. A subsequent test using an auxiliary power supply was performed to check the voltage withstanding capability of the HV plant, but another breakdown occurred at around 1MV. This paper describes the activity performed to identify the location of the breakdowns affecting the integrated tests. A test campaign has been devised with increased diagnostic capabilities and specific strategy conceived to trigger intentional breakdowns in specific locations and collect measurement patterns for different cases. The results of the campaign will be presented and the current understanding of the issue will be described, with a view on future tests and further improvements of diagnostics

Overview on electrical issues faced during the SPIDER experimental campaigns

SPIDER is the full-scale prototype of the ion source of the ITER Heating Neutral Beam Injector, where negative ions of Hydrogen or Deuterium are produced by a RF generated plasma and accelerated with a set of grids up to ~100 keV. The Power Supply System is composed of high voltage dc power supplies capable of handling frequent grid breakdowns, high current dc generators for the magnetic filter field and RF generators for the plasma generation. During the first 3 years of SPIDER operation different electrical issues were discovered, understood and addressed thanks to deep analyses of the experimental results supported by modelling activities. The paper gives an overview on the observed phenomena and relevant analyses to understand them, on the effectiveness of the short-term modifications provided to SPIDER to face the encountered issues and on the design principle of long-term solutions to be introduced during the currently ongoing long shutdown.Comment: 8 pages, 12 figures. Presented at SOFT 202

Can Minor Merging Account for the Size Growth of Quiescent Galaxies? New Results from the CANDELS Survey

The presence of extremely compact galaxies at z~2 and their subsequent growth in physical size has been the cause of much puzzlement. We revisit the question using deep infrared Wide Field Camera 3 data to probe the rest-frame optical structure of 935 host galaxies selected with 0.4 10^10.7 Msol using optical and near-infrared photometry in the UKIRT Ultra Deep Survey and GOODS-South fields of the CANDELS survey. At each redshift, the most compact sources are those with little or no star formation, and we find that the mean size of these systems grows by a factor of 3.5 +- 0.3 over this redshift interval. The new data are sufficiently deep to enable us to identify companions to these hosts whose stellar masses are ten times smaller, while still yielding suitably accurate photometric redshifts to define a likely physical association. By searching for faint companions around 404 quiescent hosts within a projected physical annulus 10 < R < 30 kpc/h, we estimate the minor merger rate over the redshift range 0.4 < z < 2. After correcting for contamination from projected pairs, we find that 13-18% of quiescent hosts have likely physical companions with stellar mass ratios of 0.1 or greater. Mergers of these companions will typically increase the host mass by 6+-2% per merger timescale. We estimate the minimum growth rate necessary to explain the declining abundance of compact galaxies. Using a simple model of merging motivated by recent numerical simulations, we then assess whether mergers of the faint companions with their hosts are sufficient to explain this minimal rate. We find that mergers with mass ratios > 0.1 may explain most of the size evolution observed at z >~ 1 if a relatively short merger timescale is assumed, but the rapid growth seen at higher redshift likely requires additional physical processes.Comment: Accepted to ApJ. Updated following referee report, with expanded comparisons to published mass-size and pair fraction measurements (Figs. 4 and 9

Minimal information for studies of extracellular vesicles (MISEV2023): From basic to advanced approaches

Extracellular vesicles (EVs), through their complex cargo, can reflect the state of their cell of origin and change the functions and phenotypes of other cells. These features indicate strong biomarker and therapeutic potential and have generated broad interest, as evidenced by the steady year-on-year increase in the numbers of scientific publications about EVs. Important advances have been made in EV metrology and in understanding and applying EV biology. However, hurdles remain to realising the potential of EVs in domains ranging from basic biology to clinical applications due to challenges in EV nomenclature, separation from non-vesicular extracellular particles, characterisation and functional studies. To address the challenges and opportunities in this rapidly evolving field, the International Society for Extracellular Vesicles (ISEV) updates its 'Minimal Information for Studies of Extracellular Vesicles', which was first published in 2014 and then in 2018 as MISEV2014 and MISEV2018, respectively. The goal of the current document, MISEV2023, is to provide researchers with an updated snapshot of available approaches and their advantages and limitations for production, separation and characterisation of EVs from multiple sources, including cell culture, body fluids and solid tissues. In addition to presenting the latest state of the art in basic principles of EV research, this document also covers advanced techniques and approaches that are currently expanding the boundaries of the field. MISEV2023 also includes new sections on EV release and uptake and a brief discussion of in vivo approaches to study EVs. Compiling feedback from ISEV expert task forces and more than 1000 researchers, this document conveys the current state of EV research to facilitate robust scientific discoveries and move the field forward even more rapidly

KIAA1109 Variants Are Associated with a Severe Disorder of Brain Development and Arthrogryposis.

Whole-exome and targeted sequencing of 13 individuals from 10 unrelated families with overlapping clinical manifestations identified loss-of-function and missense variants in KIAA1109 allowing delineation of an autosomal-recessive multi-system syndrome, which we suggest to name Alkuraya-Kučinskas syndrome (MIM 617822). Shared phenotypic features representing the cardinal characteristics of this syndrome combine brain atrophy with clubfoot and arthrogryposis. Affected individuals present with cerebral parenchymal underdevelopment, ranging from major cerebral parenchymal thinning with lissencephalic aspect to moderate parenchymal rarefaction, severe to mild ventriculomegaly, cerebellar hypoplasia with brainstem dysgenesis, and cardiac and ophthalmologic anomalies, such as microphthalmia and cataract. Severe loss-of-function cases were incompatible with life, whereas those individuals with milder missense variants presented with severe global developmental delay, syndactyly of 2nd and 3rd toes, and severe muscle hypotonia resulting in incapacity to stand without support. Consistent with a causative role for KIAA1109 loss-of-function/hypomorphic variants in this syndrome, knockdowns of the zebrafish orthologous gene resulted in embryos with hydrocephaly and abnormally curved notochords and overall body shape, whereas published knockouts of the fruit fly and mouse orthologous genes resulted in lethality or severe neurological defects reminiscent of the probands' features.This article is freely available via Open Access. Click on the Additional Link above to access the full-text via the publisher's site

The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients

The cause of Huntington disease (HD) is a polyglutamine repeat expansion of more than 36 units in the huntingtin protein, which is inversely correlated with the age at onset of the disease. However, additional genetic factors are believed to modify the course and the age at onset of HD. Recently, we identified the V471A polymorphism in the autophagy-related gene ATG7, a key component of the autophagy pathway that plays an important role in HD pathogenesis, to be associated with the age at onset in a large group of European Huntington disease patients. To confirm this association in a second independent patient cohort, we analysed the ATG7 V471A polymorphism in additional 1,464 European HD patients of the “REGISTRY” cohort from the European Huntington Disease Network (EHDN). In the entire REGISTRY cohort we could not confirm a modifying effect of the ATG7 V471A polymorphism. However, analysing a modifying effect of ATG7 in these REGISTRY patients and in patients of our previous HD cohort according to their ethnic origin, we identified a significant effect of the ATG7 V471A polymorphism on the HD age at onset only in the Italian population (327 patients). In these Italian patients, the polymorphism is associated with a 6-years earlier disease onset and thus seems to have an aggravating effect. We could specify the role of ATG7 as a genetic modifier for HD particularly in the Italian population. This result affirms the modifying influence of the autophagic pathway on the course of HD, but also suggests population-specific modifying mechanisms in HD pathogenesis

NIO1 experiment, a source of negative hydrogen and deuterium ions accelerated up to 60kV: commissioning and analysis of first experimental results of ion beam extraction

La tesi tratta della preparazione e della messa in servizio dell'esperimento NIO1, un acceleratore di ioni negativi d'idrogeno e deuterio e l'analisi dei primi risultati sperimentali. Saranno descritte le principali diagnostiche sviluppate ed installate su NIO1 e le eventuali problematiche connesse con questo esperiment

A Versatile Board for Event-Driven Data Acquisition

Event-driven data acquisition is used to capture information from fast transient phenomena typically requiring a high sampling speed. This is an important requirement in the ITER Neutral Beam Test Facility for the development of one of the heating systems of the ITER nuclear fusion experiment. The Red Pitaya board has been chosen for this project because of its versatility and low cost. Versatility is provided by the hosted Zynq System on Chip (SoC), which allows full configuration of the module architecture and the OpenSource architecture of Red Pitaya. Price is an important factor, because the boards are installed in a hostile environment where devices can be damaged by EMI and radiation. A flexible solution for event-driven data acquisition has been developed in the Zynq SoC and interfaced to the Linux-based embedded ARM processor. It has been successfully adopted in a variety of data acquisition applications in the test facility

Design of a System for Performing High-Voltage Holding Test Campaigns on a Mockup of MITICA Negative Ion Source

Megavolt ITER Injector and Concept Advance- ment (MITICA) is the full-size prototype of the neutral beam injector (NBI) for the ITER currently under construction at the neutral beam test facility (NBTF) (Consorzio RFX, Padova, Italy). One of the main issues of such a complex machine is to secure a stable voltage holding of the ion source\u2014accelerator complex\u2014which is biased at 121 MV and constitutes a very large (cathodic) area, with respect to the ground (the vessel). Coordinated with the experimental program of the HV test facilities in Japan (QST, Naka) and in Italy (high voltage Padova test facility, Consorzio RFX), a test campaign is foreseen for 2020\u20132021, using the real MITICA vacuum vessel and a mockup of the ion source. This mockup can constitute a simple geometry, where the MITICA ion source is represented by a sphere (cathode), connected to the high-voltage bushing. A planar electrode (anode) will cover the lower part of the beam source vessel (BSV) and will be positioned at an adjustable distance from the sphere, to assure fine control of the electrostatic configuration. The experiments will be devoted to characterize the breakdown voltage as a function of the gap length and vacuum pressure. In this article, the design activity of the electrodes to be used during the campaign is presented