52 research outputs found

    Guidance for human-system interfaces to automatic systems

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    Automation is ubiquitous in modern complex systems, and commercial nuclear- power plants are no exception. Automation is applied to a wide range of functions, including monitoring and detection, situation assessment, response planning, and response implementation. Automation has become a 'team player' supporting personnel in nearly all aspects of system operation. In light of its increasing use and importance in new- and future-plants, guidance is needed to conduct safety reviews of the operator's interface with automation. The objective of this research was to develop such guidance. We first characterized the important HFE aspects of automation, including six dimensions: Levels, functions, processes, modes, flexibility, and reliability. Next, we reviewed literature on the effects of all of these aspects of automation on human performance, and on the design of human-system interfaces (HSIs). Then, we used this technical basis established from the literature to identify general principles for human-automation interaction and to develop review guidelines. The guidelines consist of the following seven topics: Automation displays, interaction and control, automation modes, automation levels, adaptive automation, error tolerance and failure management, and HSI integration

    Uncertainty sources for measurable ocean carbonate chemistry variables

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    21 pages, 3 figures, 2 tables, supporting information https://doi.org/10.1002/lno.12477.-- Data availability statement: The GLODAPv2.2022 data product is freely available at the project website https://glodap.info/. The metadata data product is released as a supplementary .xlsx and .csv file with this submissionThe ocean carbonate system is critical to monitor because it plays a major role in regulating Earth's climate and marine ecosystems. It is monitored using a variety of measurements, and it is commonly understood that all components of seawater carbonate chemistry can be calculated when at least two carbonate system variables are measured. However, several recent studies have highlighted systematic discrepancies between calculated and directly measured carbonate chemistry variables and these discrepancies have large implications for efforts to measure and quantify the changing ocean carbon cycle. Given this, the Ocean Carbonate System Intercomparison Forum (OCSIF) was formed as a working group through the Ocean Carbon and Biogeochemistry program to coordinate and recommend research to quantify and/or reduce uncertainties and disagreements in measurable seawater carbonate system measurements and calculations, identify unknown or overlooked sources of these uncertainties, and provide recommendations for making progress on community efforts despite these uncertainties. With this paper we aim to (1) summarize recent progress toward quantifying and reducing carbonate system uncertainties; (2) advocate for research to further reduce and better quantify carbonate system measurement uncertainties; (3) present a small amount of new data, metadata, and analysis related to uncertainties in carbonate system measurements; and (4) restate and explain the rationales behind several OCSIF recommendations. We focus on open ocean carbonate chemistry, and caution that the considerations we discuss become further complicated in coastal, estuarine, and sedimentary environmentsThe Ocean Carbonate System Intercomparison Forum (OCSIF, https://www.us-ocb.org/ocean-carbonate-system-intercomparison-forum/) is a working group of subject matter experts that was established in 2019 with support from the Ocean Carbon and Biogeochemistry (OCB, us-ocb.org) Project Office, which receives funding from the National Science Foundation (NSF OCE-1850983) and the National Aeronautics and Space Administration (NASA NNX17AB17G). [...] B.R.C. thanks the Global Ocean Monitoring and Observing program of NOAA for funding the Carbon Data Management and Synthesis Program (Fund Ref. 100007298, program officer: Kathy Tedesco) and thereby supporting his involvement in OCSIF activities, as well as the funding the NA21OAR4310251 award (program officer: Virginia Selz), which supported the development and update of Supplementary Data S1. J.D.S. thanks the Global Ocean Monitoring and Observing program of NOAA (Award NA21OAR4310251), NOAA PMEL, and the University of Washington CICOES. R.J.W. acknowledges the National Science Foundation Division of Ocean Sciences (Oceans and Atmosphere [CSIRO] Canberra, Australia) and the MIT mTerra Catalyst fund. K.M.S. thanks the William and Elsie Knight Endowed Fellowship Fund for Marine Science (University of South Florida College of Marine Science), NOAA AOML, and the University of Miami CIMAS. K.L.F. thanks the St. Petersburg Downtown Partnership Fellowship in Coastal Science (University of South Florida College of Marine Science). M.I.G.-I. acknowledges the “Severo Ochoa Centre of Excellence” accreditation (CEX2019-000928-S). A.J.F. was supported by NOAA PMEL. M.Á. thanks the IEO internal project RADPROF. W.-J.C., R.A.E., and X.L. thank NOAA's Ocean Acidification Program via award: NA17OAR0170332. W.-J.C. also thanks NSF for support (EPSCoR-1757353 and OCE-2123768UW). [...] This research was carried out in part under the auspices of the Cooperative Institute for Climate, Ocean, and Ecosystem Studies (CICOES) and the Cooperative Institute for Marine and Atmospheric Studies (CIMAS), cooperative agreements NA20OAR4320271 and NA20OAR4320472, respectivelyPeer reviewe

    Bi-allelic loss-of-function variants in BCAS3 cause a syndromic neurodevelopmental disorder.

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    BCAS3 microtubule-associated cell migration factor (BCAS3) is a large, highly conserved cytoskeletal protein previously proposed to be critical in angiogenesis and implicated in human embryogenesis and tumorigenesis. Here, we established BCAS3 loss-of-function variants as causative for a neurodevelopmental disorder. We report 15 individuals from eight unrelated families with germline bi-allelic loss-of-function variants in BCAS3. All probands share a global developmental delay accompanied by pyramidal tract involvement, microcephaly, short stature, strabismus, dysmorphic facial features, and seizures. The human phenotype is less severe compared with the Bcas3 knockout mouse model and cannot be explained by angiogenic defects alone. Consistent with being loss-of-function alleles, we observed absence of BCAS3 in probands' primary fibroblasts. By comparing the transcriptomic and proteomic data based on probands' fibroblasts with those of the knockout mouse model, we identified similar dysregulated pathways resulting from over-representation analysis, while the dysregulation of some proposed key interactors could not be confirmed. Together with the results from a tissue-specific Drosophila loss-of-function model, we demonstrate a vital role for BCAS3 in neural tissue development

    Surface Plasmons and Surface Enhanced Raman Spectra of Aggregated and Alloyed Gold-Silver Nanoparticles

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    Effects of size, morphology, and composition of gold and silver nanoparticles on surface plasmon resonance (SPR) and surface enhanced Raman spectroscopy (SERS) are studied with the purpose of optimizing SERS substrates. Various gold and silver films made by evaporation and subsequent annealing give different morphologies and compositions of nanoparticles and thus different position of the SPR peak. SERS measurements of 4-mercaptobenzoic acid obtained from these films reveal that the proximity of the SPR peak to the exciting laser wavelength is not the only factor leading to the highest Raman enhancement. Silver nanoparticles evaporated on top of larger gold nanoparticles show higher SERS than gold-silver alloyed nanoparticles, in spite of the fact that the SPR peak of alloyed nanoparticles is narrower and closer to the excitation wavelength. The highest Raman enhancement was obtained for substrates with a two-peak particle size distribution for excitation wavelengths close to the SPR

    Dihydropyrimidine Dehydrogenase Deficiency: Metabolic Disease or Biochemical Phenotype?

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    Dihydropyrimidine dehydrogenase (DPD) deficiency is an autosomal recessive disorder of pyrimidine metabolism that impairs the first step of uracil und thymine degradation. The spectrum of clinical presentations in subjects with the full biochemical phenotype of DPD deficiency ranges from asymptomatic individuals to severely affected patients suffering from seizures, microcephaly, muscular hypotonia, developmental delay and eye abnormalities.We report on a boy with intellectual disability, significant impairment of speech development, highly active epileptiform discharges on EEG, microcephaly and impaired gross-motor development. This clinical presentation triggered metabolic workup that demonstrated the biochemical phenotype of DPD deficiency, which was confirmed by enzymatic and molecular genetic studies. The patient proved to be homozygous for a novel c.2059-22T>G mutation which resulted in an in-frame insertion of 21 base pairs (c.2059-21_c.2059-1) of intron 16 of DPYD. Family investigation showed that the asymptomatic father was also homozygous for the same mutation and enzymatic and biochemical findings were similar to his severely affected son. When the child deteriorated clinically, exome sequencing was initiated under the hypothesis that DPD deficiency did not explain the phenotype completely. A deletion of the maternal allele on chromosome 15q11.2-13-1 was identified allowing the diagnosis of Angelman syndrome (AS). This diagnosis explains the patient's clinical presentation sufficiently; the influence of DPD deficiency on the phenotype, however, remains uncertain
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