Status and Plans for the Array Control and Data Acquisition System of the Cherenkov Telescope Array

The Cherenkov Telescope Array (CTA) is the next-generation atmospheric Cherenkov gamma-ray observatory. CTA will consist of two installations, one in the northern, and the other in the southern hemisphere, containing tens of telescopes of different sizes. The CTA performance requirements and the inherent complexity associated with the operation, control and monitoring of such a large distributed multi-telescope array leads to new challenges in the field of the gamma-ray astronomy. The ACTL (array control and data acquisition) system will consist of the hardware and software that is necessary to control and monitor the CTA arrays, as well as to time-stamp, read-out, filter and store -at aggregated rates of few GB/s- the scientific data. The ACTL system must be flexible enough to permit the simultaneous automatic operation of multiple sub-arrays of telescopes with a minimum personnel effort on site. One of the challenges of the system is to provide a reliable integration of the control of a large and heterogeneous set of devices. Moreover, the system is required to be ready to adapt the observation schedule, on timescales of a few tens of seconds, to account for changing environmental conditions or to prioritize incoming scientific alerts from time-critical transient phenomena such as gamma ray bursts. This contribution provides a summary of the main design choices and plans for building the ACTL system.Comment: In Proceedings of the 34th International Cosmic Ray Conference (ICRC2015), The Hague, The Netherlands. All CTA contributions at arXiv:1508.0589

The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and family studies

Acessível em: www.ncbi.nlm.nih.gov/pmc/articles/PMC4423738/Lysosomal α-galactosidase A (α-Gal) is the enzyme deficient in Fabry disease (FD), an X-linked glycosphingolipidosis caused by pathogenic mutations affecting the GLA gene. The early-onset, multi-systemic FD classical phenotype is associated with absent or severe enzyme deficiency, as measured by in vitro assays, but patients with higher levels of residual α-Gal activity may have later-onset, more organ-restricted clinical presentations. A change in the codon 118 of the wild-type α-Gal sequence, replacing basic arginine by a potentially sulfhydryl-binding cysteine residue - GLA p.(Arg118Cys) -, has been recurrently described in large FD screening studies of high-risk patients. Although the Cys118 allele is associated with high residual α-Gal activity in vitro, it has been classified as a pathogenic mutation, mainly on the basis of theoretical arguments about the chemistry of the cysteine residue. However its pathogenicity has never been convincingly demonstrated by pathology criteria. We reviewed the clinical, biochemical and histopathology data obtained from 22 individuals of Portuguese and Spanish ancestry carrying the Cys118 allele, including 3 homozygous females. Cases were identified either on the differential diagnosis of possible FD manifestations and on case-finding studies (n=11; 4 males), or on unbiased cascade screening of probands' close relatives (n=11; 3 males). Overall, those data strongly suggest that the GLA p.(Arg118Cys) variant does not segregate with FD clinical phenotypes in a Mendelian fashion, but might be a modulator of the multifactorial risk of cerebrovascular disease. The Cys118 allelic frequency in healthy Portuguese adults (n=696) has been estimated as 0.001, therefore not qualifying for "rare" condition

Status and plans for the Array Control and Data Acquisition System of the Cherenkov Telescope Array

The Cherenkov Telescope Array (CTA) is the next-generation atmospheric Cherenkov gamma-ray observatory. CTA will consist of two installations, one in the northern, and the other in the southern hemisphere, containing tens of telescopes of different sizes. The CTA performance requirements and the inherent complexity associated with the operation, control and monitoring of such a large distributed multi-telescope array leads to new challenges in the field of the gamma-ray astronomy. The ACTL (array control and data acquisition) system will consist of the hardware and software that is necessary to control and monitor the CTA arrays, as well as to time-stamp, read-out, filter and store -at aggregated rates of few GB/s- the scientific data. The ACTL system must be flexible enough to permit the simultaneous automatic operation of multiple sub-arrays of telescopes with a minimum personnel effort on site. One of the challenges of the system is to provide a reliable integration of the control of a large and heterogeneous set of devices. Moreover, the system is required to be ready to adapt the observation schedule, on timescales of a few tens of seconds, to account for changing environmental conditions or to prioritize incoming scientific alerts from time-critical transient phenomena such as gamma ray bursts. This contribution provides a summary of the main design choices and plans for building the ACTL system

The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and family studies

Lysosomal α-galactosidase A (α-Gal) is the enzyme deficient in Fabry disease (FD), an X-linked glycosphingolipidosis caused by pathogenic mutations affecting the GLA gene. The early-onset, multi-systemic FD classical phenotype is associated with absent or severe enzyme deficiency, as measured by in vitro assays, but patients with higher levels of residual α-Gal activity may have later-onset, more organ-restricted clinical presentations. A change in the codon 118 of the wild-type α-Gal sequence, replacing basic arginine by a potentially sulfhydryl-binding cysteine residue – GLA p.(Arg118Cys) –, has been recurrently described in large FD screening studies of high-risk patients. Although the Cys118 allele is associated with high residual α-Gal activity in vitro, it has been classified as a pathogenic mutation, mainly on the basis of theoretical arguments about the chemistry of the cysteine residue. However its pathogenicity has never been convincingly demonstrated by pathology criteria. We reviewed the clinical, biochemical and histopathology data obtained from 22 individuals of Portuguese and Spanish ancestry carrying the Cys118 allele, including 3 homozygous females. Cases were identified either on the differential diagnosis of possible FD manifestations and on case-finding studies (n=11; 4 males), or on unbiased cascade screening of probands’ close relatives (n=11; 3 males). Overall, those data strongly suggest that the GLA p.(Arg118Cys) variant does not segregate with FD clinical phenotypes in a Mendelian fashion, but might be a modulator of the multifactorial risk of cerebrovascular disease, since the allelic frequency in stroke patients was 0.0087 (p=0.0185 vs the general population). The Cys118 allelic frequency in healthy Portuguese adults (n=696) has been estimated as 0.001, therefore not qualifying for “rare” conditio

Control d'Ailanthus altissima al Parc de Collserola

This document is the Final project of the Bachelor Degree in Agricultural Engineering of the Barcelona School of Agricultural Engineering , and is focused on the study of the control of the Tree of heaven (Ailanthus altissima). It includes three herbicide trials to determine a better combination, starting from a mixture used by the Serra de Collserola Natural Park. Germination test and test with rhizome fragments are also presented to evaluate their ability to resprout. The document is divided in two parts, where on the first one there is a bibliographic compilation about the Tree of heaven, and on the second one the experimental part is presented, and the development and results of the experiments can be consulted.Aquest document és el Treball de final de grau del Grau en Enginyeria Agrícola de l'Escola Superior d'Agricultura de Barcelona, i està enfocat en l'estudi del control de l'ailant (Ailanthus altíssima). Inclou tres assajos amb herbicides per a determinar una millor combinació, partint d'una mescla utilitzada pel Parc Natural de la Serra de Collserola. També es presenten assajos de germinació i assajos amb fragments de rizoma per avaluar la seva capacitat de rebrot. El document està dividit en dues parts, on a la primera hi ha recull bibliogràfic de l'ailant i a la segona s'hi presenta la part experimental, i si pot consultar el desenvolupament i resultats dels experiments.Este documento es el Trabajo de final de grado del Grado en Ingeniería Agrícola de la Escuela Superior de Agricultura de Barcelona, y esta enfocado al estudio del control del ailanto (Ailanthus altíssima). Incluye tres ensayos con herbicidas para determinar una mejor combinación, partiendo de una mezcla usada por el Parque Natural de la Sierra de Collserola. También se presentan ensayos de germinación y ensayos con fragmentos de rizoma para evaluar su capacidad de rebrote. El documento esta dividido en dos partes, donde en la primera hay una compilación bibliográfica sobre el ailanto y en la segunda se presenta la parte experimental, y se puede consultar el desarrollo y resultados de los experimentos

Control d'Ailanthus altissima al Parc de Collserola

This document is the Final project of the Bachelor Degree in Agricultural Engineering of the Barcelona School of Agricultural Engineering , and is focused on the study of the control of the Tree of heaven (Ailanthus altissima). It includes three herbicide trials to determine a better combination, starting from a mixture used by the Serra de Collserola Natural Park. Germination test and test with rhizome fragments are also presented to evaluate their ability to resprout. The document is divided in two parts, where on the first one there is a bibliographic compilation about the Tree of heaven, and on the second one the experimental part is presented, and the development and results of the experiments can be consulted.Aquest document és el Treball de final de grau del Grau en Enginyeria Agrícola de l'Escola Superior d'Agricultura de Barcelona, i està enfocat en l'estudi del control de l'ailant (Ailanthus altíssima). Inclou tres assajos amb herbicides per a determinar una millor combinació, partint d'una mescla utilitzada pel Parc Natural de la Serra de Collserola. També es presenten assajos de germinació i assajos amb fragments de rizoma per avaluar la seva capacitat de rebrot. El document està dividit en dues parts, on a la primera hi ha recull bibliogràfic de l'ailant i a la segona s'hi presenta la part experimental, i si pot consultar el desenvolupament i resultats dels experiments.Este documento es el Trabajo de final de grado del Grado en Ingeniería Agrícola de la Escuela Superior de Agricultura de Barcelona, y esta enfocado al estudio del control del ailanto (Ailanthus altíssima). Incluye tres ensayos con herbicidas para determinar una mejor combinación, partiendo de una mezcla usada por el Parque Natural de la Sierra de Collserola. También se presentan ensayos de germinación y ensayos con fragmentos de rizoma para evaluar su capacidad de rebrote. El documento esta dividido en dos partes, donde en la primera hay una compilación bibliográfica sobre el ailanto y en la segunda se presenta la parte experimental, y se puede consultar el desarrollo y resultados de los experimentos

Control d'Ailanthus altissima al Parc de Collserola

This document is the Final project of the Bachelor Degree in Agricultural Engineering of the Barcelona School of Agricultural Engineering , and is focused on the study of the control of the Tree of heaven (Ailanthus altissima). It includes three herbicide trials to determine a better combination, starting from a mixture used by the Serra de Collserola Natural Park. Germination test and test with rhizome fragments are also presented to evaluate their ability to resprout. The document is divided in two parts, where on the first one there is a bibliographic compilation about the Tree of heaven, and on the second one the experimental part is presented, and the development and results of the experiments can be consulted.Aquest document és el Treball de final de grau del Grau en Enginyeria Agrícola de l'Escola Superior d'Agricultura de Barcelona, i està enfocat en l'estudi del control de l'ailant (Ailanthus altíssima). Inclou tres assajos amb herbicides per a determinar una millor combinació, partint d'una mescla utilitzada pel Parc Natural de la Serra de Collserola. També es presenten assajos de germinació i assajos amb fragments de rizoma per avaluar la seva capacitat de rebrot. El document està dividit en dues parts, on a la primera hi ha recull bibliogràfic de l'ailant i a la segona s'hi presenta la part experimental, i si pot consultar el desenvolupament i resultats dels experiments.Este documento es el Trabajo de final de grado del Grado en Ingeniería Agrícola de la Escuela Superior de Agricultura de Barcelona, y esta enfocado al estudio del control del ailanto (Ailanthus altíssima). Incluye tres ensayos con herbicidas para determinar una mejor combinación, partiendo de una mezcla usada por el Parque Natural de la Sierra de Collserola. También se presentan ensayos de germinación y ensayos con fragmentos de rizoma para evaluar su capacidad de rebrote. El documento esta dividido en dos partes, donde en la primera hay una compilación bibliográfica sobre el ailanto y en la segunda se presenta la parte experimental, y se puede consultar el desarrollo y resultados de los experimentos

Utility of the Cobas ® Plasma Separation Card as a Sample Collection Device for Serological and Virological Diagnosis of Hepatitis C Virus Infection

Diagnosis and clinical management of people infected with hepatitis C virus (HCV) relies on results from a combination of serological and virological tests. The aim of this study was to compare the performance of dried plasma spots (DPS), prepared using the cobas ® Plasma Separation Card (PSC), to plasma and serum from venipuncture, for HCV diagnosis. We carried out a prospective study using DPS and paired plasma or serum samples. Serum and DPS samples were analyzed by immunoassay using Elecsys ® Anti-HCV II (Roche). Plasma and DPS samples were analyzed using the cobas ® HCV viral load and cobas ® HCV genotyping tests (Roche). All DPS samples that had high anti-HCV antibody titers in serum were also antibody-positive, as were five of eight samples with moderate titers. Eight samples with low titers in serum were negative with DPS. Among 80 samples with plasma HCV viral loads between 61.5 and 2.2 × 10 8 IU/mL, 74 were RNA-positive in DPS. The mean viral load difference between plasma and DPS was 2.65 log IU/mL. The performance of DPS for detection of serological and virological markers of hepatitis C virus infection was comparable to that of the conventional specimen types. However, the limits of detection were higher for DPS

Identification of an Immune-specific Class of Hepatocellular Carcinoma, Based on Molecular Features

BACKGROUND & AIMS: Agents that induce an immune response against tumors by altering T-cell regulation have increased survival times of patients with advanced-stage tumors, such as melanoma or lung cancer. We aimed to characterize molecular features of immune cells that infiltrate hepatocellular carcinomas (HCCs) to determine whether these types of agents might be effective against liver tumors. METHODS: We analyzed HCC samples from 956 patients. We separated gene expression profiles from tumor, stromal, and immune cells using a non-negative matrix factorization algorithm. We then analyzed the gene expression pattern of inflammatory cells in HCC tumor samples. We correlated expression patterns with the presence of immune cell infiltrates and immune regulatory molecules, determined by pathology and immunohistochemical analyses, in a training set of 228 HCC samples. We validated the correlation in a validation set of 728 tumor samples. Using data from 190 tumors in the Cancer Genome Atlas, we correlated immune cell gene expression profiles with numbers of chromosomal aberrations (based on single-nucleotide polymorphism array) and mutations (exome sequence data). RESULTS: We found approximately 25% of HCCs to have markers of an inflammatory response, with high expression levels of the CD274 molecule (programmed death-ligand 1) and programmed cell death 1, markers of cytolytic activity, and fewer chromosomal aberrations. We called this group of tumors the Immune class. It contained 2 subtypes, characterized by markers of an adaptive T-cell response or exhausted immune response. The exhausted immune response subclass expressed many genes regulated by transforming growth factor beta 1 that mediate immunosuppression. We did not observe any differences in numbers of mutations or expression of tumor antigens between the immune-specific class and other HCCs. CONCLUSIONS: In an analysis of HCC samples from 956 patients, we found almost 25% to express markers of an inflammatory response. We identified 2 subclasses, characterized by adaptive or exhausted immune responses. These findings indicate that some HCCs might be susceptible to therapeutic agents designed to block the regulatory pathways in T cells, such as programmed death-ligand 1, programmed cell death 1, or transforming growth factor beta 1 inhibitors