| 1. |
- Bastard, Paul, et al.
(författare)
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Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1.
- 2021
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Ingår i: The Journal of experimental medicine. - 1540-9538. ; 218:7
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Tidskriftsartikel (refereegranskat)abstract
- Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range of autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These auto-Abs were recently reported to account for at least 10% of cases of life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients from 21 kindreds in seven countries, aged between 8 and 48 yr and infected with SARS-CoV-2 since February 2020. The 21 patients tested had auto-Abs neutralizing IFN-α subtypes and/or IFN-ω; one had anti-IFN-β and another anti-IFN-ε, but none had anti-IFN-κ. Strikingly, 19 patients (86%) were hospitalized for COVID-19 pneumonia, including 15 (68%) admitted to an intensive care unit, 11 (50%) who required mechanical ventilation, and four (18%) who died. Ambulatory disease in three patients (14%) was possibly accounted for by prior or early specific interventions. Preexisting auto-Abs neutralizing type I IFNs in APS-1 patients confer a very high risk of life-threatening COVID-19 pneumonia at any age.
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| 2. |
- Marques, André R A, et al.
(författare)
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Glucosylated cholesterol in mammalian cells and tissues: formation and degradation by multiple cellular β-glucosidases.
- 2016
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Ingår i: Journal of Lipid Research. - 1539-7262. ; 57, s. 451-463
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Tidskriftsartikel (refereegranskat)abstract
- The membrane lipid glucosylceramide (GlcCer) is continuously formed and degraded. Cells express two GlcCer-degrading β-glucosidases, GBA and GBA2, located in and outside the lysosome, respectively. Here we demonstrate that through transglucosylation both GBA and GBA2 are able to catalyze in vitro the transfer of glucosyl-moieties from GlcCer to cholesterol, and vice versa. Furthermore, the natural occurrence of 1-O-cholesteryl-β-D-glucopyranoside (GlcChol) in mouse tissues and human plasma is demonstrated using LC-MS/MS and 13C6-labelled GlcChol as internal standard. In cells the inhibition of GBA increases GlcChol, whereas inhibition of GBA2 decreases glucosylated sterol. Similarly, in GBA2-deficient mice GlcChol is reduced. Depletion of GlcCer by inhibition of GlcCer synthase decreases GlcChol in cells and likewise in plasma of inhibitor-treated Gaucher disease patients. In tissues of mice with Niemann-Pick type C, a condition characterized by intralysosomal accumulation of cholesterol, marked elevations in GlcChol occur as well. When lysosomal accumulation of cholesterol is induced in cultured cells, GlcChol is formed via lysosomal GBA. This illustrates that reversible transglucosylation reactions are highly dependent on local availability of suitable acceptors. In conclusion, mammalian tissues contain GlcChol formed by transglucosylation through β-glucosidases using GlcCer as donor. Our findings reveal a novel metabolic function for GlcCer.
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| 3. |
- Carl, Johannes, et al.
(författare)
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Physical Literacy in Europe: The Current State of Implementation in Research, Practice, and Policy
- 2023
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Ingår i: Journal of Exercise Science and Fitness. - : Elsevier BV. - 1728-869X .- 2226-5104. ; 21:1, s. 165-176
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Tidskriftsartikel (refereegranskat)abstract
- The holistic concept of physical literacy (PL) embraces different person-centered qualities (physical, cognitive, affective/psychological) necessary to lead physically active lifestyles. PL has recently gained increasing attention globally and Europe is no exception. However, scientific endeavors summarizing the current state of PL in Europe are lacking. Therefore, the goal of this study was to comprehensively assess and compare the implementation of PL in research, policy, and practice across the continent. We assembled a panel of experts representing 25 European countries. Employing a complementary mixed-methods design, the experts first prepared reviews about the current state of PL in their countries (categories: research, practice/policy). The reviews underwent comparative document analysis ensuring a transnational foureyes principle. For re-validation purposes, the representatives completed a quantitative survey with questions reflecting the inductive themes from the document analysis. The document analysis resulted in ten disjunct themes (related to “concept”, “research”, “practice/policy”, “future/prospect”) and yielded a heterogenous PL situation in Europe. The implementation state was strongly linked to conceptual discussions (e.g., existence of competing approaches), linguistic issues (e.g., translations), and country-specific traditions. Despite growing scholarly attention, PL hesitantly permeates practice and policy in most countries. Nevertheless, the experts largely anticipate increasing popularity of PL for the future. Despite the heterogeneous situation across Europe, the analysis has uncovered similarities among the countries, such as the presence of established yet not identical concept. Research should intensify academic activities (conceptuallinguistic elaborations, empirical work) before PL may gain further access into practical and political spheres in the long term.
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| 4. |
- Kisser, Johannes, et al.
(författare)
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A review of nature-based solutions for resource recovery in cities
- 2020
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Ingår i: BLUE-GREEN SYSTEMS. - : IWA Publishing. - 2617-4782. ; 2:1, s. 138-172
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Forskningsöversikt (refereegranskat)abstract
- Our modern cities are resource sinks designed on the current linear economic model which recovers very little of the original input. As the current model is not sustainable, a viable solution is to recover and reuse parts of the input. In this context, resource recovery using nature-based solutions (NBS) is gaining popularity worldwide. In this specific review, we focus on NBS as technologies that bring nature into cities and those that are derived from nature, using (micro)organisms as principal agents, provided they enable resource recovery. The findings presented in this work are based on an extensive literature review, as well as on original results of recent innovation projects across Europe. The case studies were collected by participants of the COST Action Circular City, which includes a portfolio of more than 92 projects. The present review article focuses on urban wastewater, industrial wastewater, municipal solid waste and gaseous effluents, the recoverable products (e.g., nutrients, nanoparticles, energy), as well as the implications of source-separation and circularity by design. The analysis also includes assessment of the maturity of different technologies (technology readiness level) and the barriers that need to be overcome to accelerate the transition to resilient, self-sustainable cities of the future.
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| 5. |
- Winberg von Friesen, Lisa, et al.
(författare)
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Summer sea ice melt and wastewater are important local sources of microlitter to Svalbard waters
- 2020
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Ingår i: Environment International. - : Elsevier BV. - 0160-4120 .- 1873-6750. ; 139
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Tidskriftsartikel (refereegranskat)abstract
- Human activities leave traces of marine litter around the globe. The Arctic is, despite its remoteness, emerging as an area of no exception to this environmental issue. Arctic sea ice has previously been found to constitute a temporal sink of microplastics, but the potential release and subsequent fate of microplastics in the marine environment are yet unknown. Furthermore, the relative importance of local sources of microplastics in the Arctic marine environment is under discussion. In this study, the concentration and distribution of anthropogenic microparticles (AMPs, <5 mm, including microplastics) have been investigated in marine waters and sea ice of Svalbard. Seawater samples throughout the water column and floating sea ice samples were collected along a transect originating in Rijpfjorden, reaching northwards to the sea ice-edge. Seawater samples were also collected along a transect extending westwards from head to mouth of Kongsfjorden. Samples were collected throughout the water column with stations positioned to enable detection of potential AMP emissions from the wastewater outlet in Ny-Ålesund. Along both transects, environmental parameters were measured to explore potential correlations with AMP distribution. High concentrations of AMPs were detected in sea ice (158 ± 155 AMPs L−1). Based on both AMP concentrations and characteristics, AMPs identified in seawater of the marginal ice zone are to a large extent likely released during the melting of sea ice. The release of AMPs during summer melting of sea ice was concomitantly taking place with the ice-edge bloom, suggesting increased bioavailability to Arctic marine biota. Concentrations of AMPs were up to an order of magnitude higher in Kongsfjorden (up to 48.0 AMPs L−1) than in Rijpfjorden (up to 7.4 AMPs L−1). The distribution and composition of AMPs in Kongsfjorden suggest the wastewater outlet in Ny-Ålesund to be a likely source. Our results emphasize the importance of local point- and diffuse sources of AMPs in the Arctic and stress the urgency of considering their associated environmental impact. Implementation of regulatory policy is of importance, particularly since human activities and environmental pressures are increasing in the Arctic. © 2020 The Authors
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