Forward Diffractive Detector control system for Run 3 in the ALICE experiment Mejía Camacho, J.M.; Rodríguez Ramírez, S.A.; Cabanillas Noris, J.C. ...
Nuclear instruments & methods in physics research. Section A, Accelerators, spectrometers, detectors and associated equipment,
05/2023, Letnik:
1050
Journal Article
Recenzirano
The ALICE experiment went through major upgrades in preparation for Runs 3 and 4 at the CERN LHC, which foresee a significant increase in the data stream rate. To cope with it, a new Computing System ...Online–Offline (O2) was developed by ALICE and several detectors were upgraded. One of these upgrades corresponded to the ALICE Diffractive (AD) detector, which was replaced by the new Forward Diffractive Detector (FDD). A fundamental system that allows for recording of data as well as for the stable and safe operation of the experimental setup is the Detector Control System (DCS). The DCS controls, monitors, and configures detectors’ hardware and their subsystems among which are commercial hardware and specific custom equipment. In this work, we describe the implementation of the DCS of FDD, which was designed and developed using the SCADA commercial software WinCC Open Architecture (WinCC-OA®) and the Joint Controls Project (JCOP) Framework. We also describe the modeling of FDD-DCS as a finite state machine to be integrated into the ALICE Central DCS, and to be operated by DCS shifters in the run control center and, remotely, by detector experts. This new DCS was developed to comply with the new LHC operational standards that allow for processing the increased luminosity due to collisions at a higher energy, and for fulfilling the specific requirements of the ALICE experiment, in order to address the scientific challenges of its physics program.
The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent ...complement C3 deposition in kidney biopsy samples. The two major subgroups of C3 glomerulopathy - dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) - have overlapping clinical and pathological features suggestive of a disease continuum. Dysregulation of the complement alternative pathway is fundamental to the manifestations of C3 glomerulopathy, although terminal pathway dysregulation is also common. Disease is driven by acquired factors in most patients - namely, autoantibodies that target the C3 or C5 convertases. These autoantibodies drive complement dysregulation by increasing the half-life of these vital but normally short-lived enzymes. Genetic variation in complement-related genes is a less frequent cause. No disease-specific treatments are available, although immunosuppressive agents and terminal complement pathway blockers are helpful in some patients. Unfortunately, no treatment is universally effective or curative. In aggregate, the limited data on renal transplantation point to a high risk of disease recurrence (both DDD and C3GN) in allograft recipients. Clinical trials are underway to test the efficacy of several first-generation drugs that target the alternative complement pathway.
Metal hydrides are known as a potential efficient, low-risk option for high-density hydrogen storage since the late 1970s. In this paper, the present status and the future perspectives of the use of ...metal hydrides for hydrogen storage are discussed. Since the early 1990s, interstitial metal hydrides are known as base materials for Ni – metal hydride rechargeable batteries. For hydrogen storage, metal hydride systems have been developed in the 2010s 1 for use in emergency or backup power units, i. e. for stationary applications.
With the development and completion of the first submarines of the U212 A series by HDW (now Thyssen Krupp Marine Systems) in 2003 and its export class U214 in 2004, the use of metal hydrides for hydrogen storage in mobile applications has been established, with new application fields coming into focus.
In the last decades, a huge number of new intermetallic and partially covalent hydrogen absorbing compounds has been identified and partly more, partly less extensively characterized.
In addition, based on the thermodynamic properties of metal hydrides, this class of materials gives the opportunity to develop a new hydrogen compression technology. They allow the direct conversion from thermal energy into the compression of hydrogen gas without the need of any moving parts. Such compressors have been developed and are nowadays commercially available for pressures up to 200 bar. Metal hydride based compressors for higher pressures are under development. Moreover, storage systems consisting of the combination of metal hydrides and high-pressure vessels have been proposed as a realistic solution for on-board hydrogen storage on fuel cell vehicles.
In the frame of the “Hydrogen Storage Systems for Mobile and Stationary Applications” Group in the International Energy Agency (IEA) Hydrogen Task 32 “Hydrogen-based energy storage”, different compounds have been and will be scaled-up in the near future and tested in the range of 500 g to several hundred kg for use in hydrogen storage applications.
•Latest developments in the application of hydride technology.•Investigations on both stationary and mobile applications.•Special attention to metal hydride compressors.•Heat storage and H2 compression especially noted in the Outlook.
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving ...Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.
The pathogenesis of atypical hemolytic uremic syndrome (aHUS) is strongly linked to dysregulation of the alternative pathway of the complement system. Mutations in complement genes have been ...identified in about two-thirds of cases, with 5% to 15% being in C3. In this study, 23 aHUS-associated genetic changes in C3 were characterized relative to their interaction with the control proteins factor H (FH), membrane cofactor protein (MCP; CD46), and complement receptor 1 (CR1; CD35). In surface plasmon resonance experiments, 17 mutant recombinant proteins demonstrated a defect in binding to FH and/or MCP, whereas 2 demonstrated reduced binding to CR1. In the majority of cases, decreased binding affinity translated to a decrease in proteolytic inactivation (known as cofactor activity) of C3b via FH and MCP. These results were used to map the putative binding regions of C3b involved in the interaction with MCP and CR1 and interrogated relative to known FH binding sites. Seventy-six percent of patients with C3 mutations had low C3 levels that correlated with disease severity. This study expands our knowledge of the functional consequences of aHUS-associated C3 mutations relative to the interaction of C3 with complement regulatory proteins mediating cofactor activity.
•C3 mutations in aHUS commonly result in impaired complement regulation, C3 consumption, and a poor renal outcome.•C3 mutations tend to cluster at the protein surface and facilitate mapping of putative binding sites for the regulatory proteins.
PM2.5 exposure is associated with significant health risk. Exposures in homes derive from both outdoor and indoor sources, with emissions occurring primarily in discrete events. Data on emission ...event magnitudes and schedules are needed to support simulation‐based studies of exposures and mitigations. This study applied an identification and characterization algorithm to quantify time‐resolved PM2.5 emission events from data collected during 224 days of monitoring in 18 California apartments with low‐income residents. We identified and characterized 836 distinct events with median and mean values of 12 and 30 mg emitted mass, 16 and 23 minutes emission duration, 37 and 103 mg/h emission rates, and pseudo‐first–order decay rates of 1.3 and 2.0/h. Mean event‐averaged concentrations calculated using the determined event characteristics agreed to within 6% of measured values for 14 of the apartments. There were variations in event schedules and emitted mass across homes, with few events overnight and most emissions occurring during late afternoons and evenings. Event characteristics were similar during weekdays and weekends. Emitted mass was positively correlated with number of residents (Spearman coefficient, ρ=.10), bedrooms (ρ=.08), house volume (ρ=.29), and indoor‐outdoor CO2 difference (ρ=.27). The event schedules can be used in probabilistic modeling of PM2.5 in low‐income apartments.
The safety and efficacy of an attenuated G1P8 human rotavirus (HRV) vaccine were tested in a randomized, double-blind, phase 3 trial.
We studied 63,225 healthy infants from 11 Latin American ...countries and Finland who received two oral doses of either the HRV vaccine (31,673 infants) or placebo (31,552 infants) at approximately two months and four months of age. Severe gastroenteritis episodes were identified by active surveillance. The severity of disease was graded with the use of the 20-point Vesikari scale. Vaccine efficacy was evaluated in a subgroup of 20,169 infants (10,159 vaccinees and 10,010 placebo recipients).
The efficacy of the vaccine against severe rotavirus gastroenteritis and against rotavirus-associated hospitalization was 85 percent (P<0.001 for the comparison with placebo) and reached 100 percent against more severe rotavirus gastroenteritis. Hospitalization for diarrhea of any cause was reduced by 42 percent (95 percent confidence interval, 29 to 53 percent; P<0.001). During the 31-day window after each dose, six vaccine recipients and seven placebo recipients had definite intussusception (difference in risk, -0.32 per 10,000 infants; 95 percent confidence interval, -2.91 to 2.18; P=0.78).
Two oral doses of the live attenuated G1P8 HRV vaccine were highly efficacious in protecting infants against severe rotavirus gastroenteritis, significantly reduced the rate of severe gastroenteritis from any cause, and were not associated with an increased risk of intussusception. (ClinicalTrials.gov numbers, NCT00139347 and NCT00263666.)
Summary
Background
Single nucleotide polymorphisms (SNPs) in platelet‐associated genes partly explain inherent variability in platelet counts. Patients with monoallelic Bernard Soulier syndrome due ...to the Bolzano mutation (GPIBA A156V) have variable platelet counts despite a common mutation for unknown reasons.
Objectives
We investigated the effect of the most common SNP (R307H) in the hematopoietic‐specific tubulin isotype β‐1 in these Bernard Soulier patients and potential microtubule‐based mechanisms of worsened thrombocytopenia.
Patients/Methods
Ninety‐four monoallelic Bolzano mutation patients were evaluated for the R307H β‐1 SNP and had platelet counts measured by three methods; the Q43P SNP was also evaluated. To investigate possible mechanisms underlying this association, we used molecular modeling of β‐1 tubulin with and without the R307H SNP. We transfected SNP or non‐SNP β‐1 tubulin into MCF‐7 and CMK cell lines and measured microtubule regrowth after nocodazole‐induced depolymerization.
Results
We found that patients with at least one R307H SNP allele had significantly worse thrombocytopenia; manual platelet counting revealed a median platelet count of 124 in non‐SNP patients and 76 in SNP patients (both ×109 L−1; P < 0.01). The Q43P SNP had no significant association with platelet count. Molecular modeling suggested a structural relationship between the R307H SNP and microtubule stability via alterations in the M‐loop of β tubulin; in vitro microtubule recovery assays revealed that cells transfected with R307H SNP β‐1 had significantly impaired microtubule recovery.
Conclusions
Our data show that the R307H SNP is significantly associated with the degree of thrombocytopenia in congenital and acquired platelet disorders, and may affect platelets by altering microtubule behavior.
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