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Träfflista för sökning "WFRF:(Müller Christoph) srt2:(2015-2019)"

Sökning: WFRF:(Müller Christoph) > (2015-2019)

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1.
  • Kehoe, Laura, et al. (författare)
  • Make EU trade with Brazil sustainable
  • 2019
  • Ingår i: Science. - : American Association for the Advancement of Science (AAAS). - 0036-8075 .- 1095-9203. ; 364:6438, s. 341-
  • Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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  • Schewe, Jacob, et al. (författare)
  • State-of-the-art global models underestimate impacts from climate extremes
  • 2019
  • Ingår i: Nature Communications. - : Springer Science and Business Media LLC. - 2041-1723. ; 10
  • Tidskriftsartikel (refereegranskat)abstract
    • Global impact models represent process-level understanding of how natural and human systems may be affected by climate change. Their projections are used in integrated assessments of climate change. Here we test, for the first time, systematically across many important systems, how well such impact models capture the impacts of extreme climate conditions. Using the 2003 European heat wave and drought as a historical analogue for comparable events in the future, we find that a majority of models underestimate the extremeness of impacts in important sectors such as agriculture, terrestrial ecosystems, and heat-related human mortality, while impacts on water resources and hydropower are overestimated in some river basins; and the spread across models is often large. This has important implications for economic assessments of climate change impacts that rely on these models. It also means that societal risks from future extreme events may be greater than previously thought.
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4.
  • Wartenburger, Richard, et al. (författare)
  • Evapotranspiration simulations in ISIMIP2a-Evaluation of spatio-temporal characteristics with a comprehensive ensemble of independent datasets
  • 2018
  • Ingår i: Environmental Research Letters. - : IOP Publishing. - 1748-9326. ; 13:7
  • Tidskriftsartikel (refereegranskat)abstract
    • Actual land evapotranspiration (ET) is a key component of the global hydrological cycle and an essential variable determining the evolution of hydrological extreme events under different climate change scenarios. However, recently available ET products show persistent uncertainties that are impeding a precise attribution of human-induced climate change. Here, we aim at comparing a range of independent global monthly land ET estimates with historical model simulations from the global water, agriculture, and biomes sectors participating in the second phase of the Inter-Sectoral Impact Model Intercomparison Project (ISIMIP2a). Among the independent estimates, we use the EartH2Observe Tier-1 dataset (E2O), two commonly used reanalyses, a pre-compiled ensemble product (LandFlux-EVAL), and an updated collection of recently published datasets that algorithmically derive ET from observations or observations-based estimates (diagnostic datasets). A cluster analysis is applied in order to identify spatio-temporal differences among all datasets and to thus identify factors that dominate overall uncertainties. The clustering is controlled by several factors including the model choice, the meteorological forcing used to drive the assessed models, the data category (models participating in the different sectors of ISIMIP2a, E2O models, diagnostic estimates, reanalysis-based estimates or composite products), the ET scheme, and the number of soil layers in the models. By using these factors to explain spatial and spatio-temporal variabilities in ET, we find that the model choice mostly dominates (24%-40% of variance explained), except for spatio-temporal patterns of total ET, where the forcing explains the largest fraction of the variance (29%). The most dominant clusters of datasets are further compared with individual diagnostic and reanalysis-based estimates to assess their representation of selected heat waves and droughts in the Great Plains, Central Europe and western Russia. Although most of the ET estimates capture these extreme events, the generally large spread among the entire ensemble indicates substantial uncertainties.
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5.
  • Andresen, Louise C., 1974, et al. (författare)
  • Shifting Impacts of Climate Change: Long-Term Patterns of Plant Response to Elevated CO2, Drought, and Warming Across Ecosystems
  • 2016
  • Ingår i: Large-Scale Ecology: Model Systems to Global Perspectives. - : Elsevier. - 9780081009352 ; , s. 437-473
  • Bokkapitel (refereegranskat)abstract
    • Field experiments that expose terrestrial ecosystems to climate change factors by manipulations are expensive to maintain, and typically only last a few years. Plant biomass is commonly used to assess responses to climate treatments and to predict climate change impacts. However, response to the treatments might be considerably different between the early years and a decade later. The aim of this data analysis was to develop and apply a method for evaluating changes in plant biomass responses through time, in order to provide a firm basis for discussing how the ‘short-term’ response might differ from the ‘long-term’ response. Across 22 sites situated in the northern hemisphere, which covered three continents, and multiple ecosystems (grasslands, shrublands, moorlands, forests, and deserts), we evaluated biomass datasets from long-term experiments with exposure to elevated CO2 (eCO2), warming, or drought. We developed methods for assessing biomass response patterns to the manipulations using polynomial and linear (piecewise) model analysis and linked the responses to sitespecific variables such as temperature and rainfall. Polynomial patterns across sites indicated changes in response direction over time under eCO2, warming, and drought. In addition, five distinct pattern types were confirmed within sites: ‘no response’, ‘delayed response’, ‘directional response’, ‘dampening response’, and ‘altered response’ patterns. We found that biomass response direction was as likely to change over time as it was to be consistent, and therefore suggest that climate manipulation experiments should be carried out over timescales covering both short- and long-term responses, in order to realistically assess future impacts of climate change.
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6.
  • Attié, David, et al. (författare)
  • A time projection chamber with GEM-based readout
  • 2017
  • Ingår i: Nuclear Instruments and Methods in Physics Research, Section A: Accelerators, Spectrometers, Detectors and Associated Equipment. - : Elsevier BV. - 0168-9002. ; 856, s. 109-118
  • Tidskriftsartikel (refereegranskat)abstract
    • For the International Large Detector concept at the planned International Linear Collider, the use of time projection chambers (TPC) with micro-pattern gas detector readout as the main tracking detector is investigated. In this paper, results from a prototype TPC, placed in a 1. T solenoidal field and read out with three independent Gas Electron Multiplier (GEM) based readout modules, are reported. The TPC was exposed to a 6. GeV electron beam at the DESY II synchrotron. The efficiency for reconstructing hits, the measurement of the drift velocity, the space point resolution and the control of field inhomogeneities are presented.
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8.
  • Brenner, David, et al. (författare)
  • Hot-spot KIF5A mutations cause familial ALS
  • 2018
  • Ingår i: Brain. - : Oxford University Press. - 0006-8950 .- 1460-2156. ; 141, s. 688-697
  • Tidskriftsartikel (refereegranskat)abstract
    • Heterozygous missense mutations in the N-terminal motor or coiled-coil domains of the kinesin family member 5A (KIF5A) gene cause monogenic spastic paraplegia (HSP10) and Charcot-Marie-Tooth disease type 2 (CMT2). Moreover, heterozygous de novo frame-shift mutations in the C-terminal domain of KIF5A are associated with neonatal intractable myoclonus, a neurodevelopmental syndrome. These findings, together with the observation that many of the disease genes associated with amyotrophic lateral sclerosis disrupt cytoskeletal function and intracellular transport, led us to hypothesize that mutations in KIF5A are also a cause of amyotrophic lateral sclerosis. Using whole exome sequencing followed by rare variant analysis of 426 patients with familial amyotrophic lateral sclerosis and 6137 control subjects, we detected an enrichment of KIF5A splice-site mutations in amyotrophic lateral sclerosis (2/426 compared to 0/6137 in controls; P = 4.2 x 10-3), both located in a hot-spot in the C-terminus of the protein and predicted to affect splicing exon 27. We additionally show co-segregation with amyotrophic lateral sclerosis of two canonical splice-site mutations in two families. Investigation of lymphoblast cell lines from patients with KIF5A splice-site mutations revealed the loss of mutant RNA expression and suggested haploinsufficiency as the most probable underlying molecular mechanism. Furthermore, mRNA sequencing of a rare non-synonymous missense mutation (predicting p. Arg1007Gly) located in the C-terminus of the protein shortly upstream of the splice donor of exon 27 revealed defective KIF5A pre-mRNA splicing in respective patient-derived cell lines owing to abrogation of the donor site. Finally, the non-synonymous single nucleotide variant rs113247976 (minor allele frequency = 1.00% in controls, n = 6137), also located in the C-terminal region [p.(Pro986Leu) in exon 26], was significantly enriched in familial amyotrophic lateral sclerosis patients (minor allele frequency = 3.40%; P = 1.28 x 10-7). Our study demonstrates that mutations located specifically in a C-terminal hotspot of KIF5A can cause a classical amyotrophic lateral sclerosis phenotype, and underline the involvement of intracellular transport processes in amyotrophic lateral sclerosis pathogenesis.
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9.
  • Brockmann, Sarah J., et al. (författare)
  • CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency
  • 2018
  • Ingår i: Human Molecular Genetics. - : Oxford University Press. - 0964-6906 .- 1460-2083. ; 27:4, s. 706-715
  • Tidskriftsartikel (refereegranskat)abstract
    • Mutations in the mitochondrially located protein CHCHD10 cause motoneuron disease by an unknown mechanism. In this study, we investigate the mutations p. R15L and p. G66V in comparison to wild-type CHCHD10 and the non-pathogenic variant p. P34S in vitro, in patient cells as well as in the vertebrate in vivo model zebrafish. We demonstrate a reduction of CHCHD10 protein levels in p. R15L and p. G66V mutant patient cells to approximately 50%. Quantitative real-time PCR revealed that expression of CHCHD10 p. R15L, but not of CHCHD10 p. G66V, is already abrogated at the mRNA level. Altered secondary structure and rapid protein degradation are observed with regard to the CHCHD10 p. G66V mutant. In contrast, no significant differences in expression, degradation rate or secondary structure of non-pathogenic CHCHD10 p. P34S are detected when compared with wild-type protein. Knockdown of CHCHD10 expression in zebrafish to about 50% causes motoneuron pathology, abnormal myofibrillar structure and motility deficits in vivo. Thus, our data show that the CHCHD10 mutations p. R15L and p. G66V cause motoneuron disease primarily based on haploinsufficiency of CHCHD10.
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10.
  • Brueckner, Frank, et al. (författare)
  • Enhanced manufacturing possibilities using multi-materials in laser metal deposition
  • 2018
  • Ingår i: Journal of laser applications. - : American Institute of Physics (AIP). - 1042-346X .- 1938-1387. ; 26:2, s. 10-12
  • Tidskriftsartikel (refereegranskat)abstract
    • Additive manufacturing (AM) addresses various benefits as the buildup of complex shaped parts, the possibility of functional integration, reduced lead times or the use of difficult machinable materials compared to conventional manufacturing possibilities. Beside these advantages, the use of more than one material in a component would strongly increase the field of applications in typical AM branches as energy, aerospace, or medical technology. By means of multi-material buildups, cost-intensive alloys could be only used in high-loaded areas of the part, whereas the remaining part could be fabricated with cheaper compositions. The selection of combined materials strongly depends on the requested thermophysical but also mechanical properties. Within this contribution, examples (e.g., used in the turbine business) show how alloys can be arranged to fit together, e.g., in terms of a well-chosen coefficient of thermal expansion. As can be seen in nature, the multi-material usage can be characterized by sharp intersections from one material to the other (e.g., in case of a thin corrosion protection), but also by graded structures enabling a smoother material transition (e.g., in case of dissimilar materials which are joined together without defects). The latter is shown for an example from aerospace within this paper. Another possibility is the simultaneous placement of several materials, e.g., hard carbide particles placed in a more ductile matrix composition. These particles can be varied in size (e.g., TiC versus WC). Also the ratio between carbides and matrix alloy can be adjusted depending on its application. Especially, nozzle-based free form fabrication technologies, e.g., laser metal deposition, enable the utilization of more than one material. Within this contribution, possibilities to feed more than one filler material are demonstrated. In addition, results of multi-material processes are shown. Finally, this work focuses on different (potential) applications, mainly on power generation, but also for medical technology or wear resistant components.
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