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| 2. |
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| 3. |
- Chelban, V., et al.
(författare)
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PDXK mutations cause polyneuropathy responsive to pyridoxal 5′-phosphate supplementation
- 2019
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Ingår i: Annals of Neurology. - : Wiley. - 0364-5134 .- 1531-8249. ; 86:2, s. 225-240
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To identify disease-causing variants in autosomal recessive axonal polyneuropathy with optic atrophy and provide targeted replacement therapy. Methods: We performed genome-wide sequencing, homozygosity mapping, and segregation analysis for novel disease-causing gene discovery. We used circular dichroism to show secondary structure changes and isothermal titration calorimetry to investigate the impact of variants on adenosine triphosphate (ATP) binding. Pathogenicity was further supported by enzymatic assays and mass spectroscopy on recombinant protein, patient-derived fibroblasts, plasma, and erythrocytes. Response to supplementation was measured with clinical validated rating scales, electrophysiology, and biochemical quantification. Results: We identified biallelic mutations in PDXK in 5 individuals from 2 unrelated families with primary axonal polyneuropathy and optic atrophy. The natural history of this disorder suggests that untreated, affected individuals become wheelchair-bound and blind. We identified conformational rearrangement in the mutant enzyme around the ATP-binding pocket. Low PDXK ATP binding resulted in decreased erythrocyte PDXK activity and low pyridoxal 5′-phosphate (PLP) concentrations. We rescued the clinical and biochemical profile with PLP supplementation in 1 family, improvement in power, pain, and fatigue contributing to patients regaining their ability to walk independently during the first year of PLP normalization. Interpretation: We show that mutations in PDXK cause autosomal recessive axonal peripheral polyneuropathy leading to disease via reduced PDXK enzymatic activity and low PLP. We show that the biochemical profile can be rescued with PLP supplementation associated with clinical improvement. As B6 is a cofactor in diverse essential biological pathways, our findings may have direct implications for neuropathies of unknown etiology characterized by reduced PLP levels. ANN NEUROL 2019;86:225–240. © 2019 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.
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| 5. |
- Daniau, A. -L, et al.
(författare)
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predictability of biomass burning in response to climate changes
- 2012
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Ingår i: Global Biogeochemical Cycles. - 0886-6236 .- 1944-9224. ; 26, s. GB4007-
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Tidskriftsartikel (refereegranskat)abstract
- Climate is an important control on biomass burning, but the sensitivity of fire to changes in temperature and moisture balance has not been quantified. We analyze sedimentary charcoal records to show that the changes in fire regime over the past 21,000 yrs are predictable from changes in regional climates. Analyses of paleo-fire data show that fire increases monotonically with changes in temperature and peaks at intermediate moisture levels, and that temperature is quantitatively the most important driver of changes in biomass burning over the past 21,000 yrs. Given that a similar relationship between climate drivers and fire emerges from analyses of the interannual variability in biomass burning shown by remote-sensing observations of month-by-month burnt area between 1996 and 2008, our results signal a serious cause for concern in the face of continuing global warming.
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| 7. |
- Sim, Thomas G., et al.
(författare)
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Regional variability in peatland burning at mid-to high-latitudes during the Holocene
- 2023
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Ingår i: Quaternary Science Reviews. - : Elsevier. - 0277-3791 .- 1873-457X. ; 305
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Tidskriftsartikel (refereegranskat)abstract
- Northern peatlands store globally-important amounts of carbon in the form of partly decomposed plant detritus. Drying associated with climate and land-use change may lead to increased fire frequency and severity in peatlands and the rapid loss of carbon to the atmosphere. However, our understanding of the patterns and drivers of peatland burning on an appropriate decadal to millennial timescale relies heavily on individual site-based reconstructions. For the first time, we synthesise peatland macrocharcoal re-cords from across North America, Europe, and Patagonia to reveal regional variation in peatland burning during the Holocene. We used an existing database of proximal sedimentary charcoal to represent regional burning trends in the wider landscape for each region. Long-term trends in peatland burning appear to be largely climate driven, with human activities likely having an increasing influence in the late Holocene. Warmer conditions during the Holocene Thermal Maximum (similar to 9e6 cal. ka BP) were associated with greater peatland burning in North America's Atlantic coast, southern Scandinavia and the Baltics, and Patagonia. Since the Little Ice Age, peatland burning has declined across North America and in some areas of Europe. This decline is mirrored by a decrease in wider landscape burning in some, but not all sub-regions, linked to fire-suppression policies, and landscape fragmentation caused by agricultural expansion. Peatlands demonstrate lower susceptibility to burning than the wider landscape in several instances, probably because of autogenic processes that maintain high levels of near-surface wetness even during drought. Nonetheless, widespread drying and degradation of peatlands, particularly in Europe, has likely increased their vulnerability to burning in recent centuries. Consequently, peatland restoration efforts are important to mitigate the risk of peatland fire under a changing climate. Finally, we make recommendations for future research to improve our understanding of the controls on peatland fires.(c) 2023 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
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| 8. |
- Githumbi, Esther, et al.
(författare)
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European pollen-based REVEALS land-cover reconstructions for the Holocene : Methodology, mapping and potentials
- 2022
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Ingår i: Earth System Science Data. - : Copernicus GmbH. - 1866-3508 .- 1866-3516. ; 14:4, s. 1581-1619
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Tidskriftsartikel (refereegranskat)abstract
- Quantitative reconstructions of past land cover are necessary to determine the processes involved in climate-human-land-cover interactions. We present the first temporally continuous and most spatially extensive pollen-based land-cover reconstruction for Europe over the Holocene (last 11g€¯700g€¯calg€¯yrg€¯BP). We describe how vegetation cover has been quantified from pollen records at a 11 spatial scale using the "Regional Estimates of VEgetation Abundance from Large Sites"(REVEALS) model. REVEALS calculates estimates of past regional vegetation cover in proportions or percentages. REVEALS has been applied to 1128 pollen records across Europe and part of the eastern Mediterranean-Black Sea-Caspian corridor (30-75° N, 25° W-50° E) to reconstruct the percentage cover of 31 plant taxa assigned to 12 plant functional types (PFTs) and 3 land-cover types (LCTs). A new synthesis of relative pollen productivities (RPPs) for European plant taxa was performed for this reconstruction. It includes multiple RPP values (≥2 values) for 39 taxa and single values for 15 taxa (total of 54 taxa). To illustrate this, we present distribution maps for five taxa (Calluna vulgaris, Cerealia type (t)., Picea abies, deciduous Quercus t. and evergreen Quercus t.) and three land-cover types (open land, OL; evergreen trees, ETs; and summer-green trees, STs) for eight selected time windows. The reliability of the REVEALS reconstructions and issues related to the interpretation of the results in terms of landscape openness and human-induced vegetation change are discussed. This is followed by a review of the current use of this reconstruction and its future potential utility and development. REVEALS data quality are primarily determined by pollen count data (pollen count and sample, pollen identification, and chronology) and site type and number (lake or bog, large or small, one site vs. multiple sites) used for REVEALS analysis (for each grid cell). A large number of sites with high-quality pollen count data will produce more reliable land-cover estimates with lower standard errors compared to a low number of sites with lower-quality pollen count data. The REVEALS data presented here can be downloaded from https://doi.org/10.1594/PANGAEA.937075 (Fyfe et al., 2022).
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| 10. |
- Lorenzzi, J., et al.
(författare)
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Incorporation of group III, IV and V elements in 3C–SiC(1 1 1) layers grown by the vapour–liquid–solid mechanism
- 2010
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Ingår i: Journal of Crystal Growth. - : Elsevier BV. - 0022-0248 .- 1873-5002. ; 312:23, s. 3443-3450
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Tidskriftsartikel (refereegranskat)abstract
- We report on a comparative investigation of the incorporation of group III, IV and V impurities in 3C–SiC heteroepitaxial layers grown by the vapour–liquid–solid (VLS) mechanism on on-axis α-SiC substrates. To this end, various Si-based melts have been used with addition of Al, Ga, Ge and Sn species. Homoepitaxial α-SiC layers grown using Al-based melts were used for comparison purposed for Al incorporation. Nitrogen incorporation depth profile systematically displays an overshoot at the substrate/epilayer interface for all the layers. Ga and Al incorporations follow the same distribution shape as N whereas this is not the case for the isoelectronic impurities Ge and Sn. This suggests some interaction between Ga/Al and N coming from the high bonding energy between the group III and V elements, which does not exist with Ge and Sn. This is why both incorporate as a cluster. A model of incorporation is proposed taking into account metal-N and metal-C bonding energies together with the solid solubility of the corresponding nitrides.
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| 11. |
- Marinova, Maya, et al.
(författare)
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Depth Profiling Charge Accumulation from a Ferroelectric into a Doped Mott Insulator
- 2015
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Ingår i: Nano letters (Print). - : American Chemical Society (ACS). - 1530-6984 .- 1530-6992. ; 15:4, s. 2533-2541
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Tidskriftsartikel (refereegranskat)abstract
- The electric field control of functional properties is a crucial goal in oxide-based electronics. Nonvolatile switching between different resistivity or magnetic states in an oxide channel can be achieved through charge accumulation or depletion from an adjacent ferroelectric. However, the way in which charge distributes near the interface between the ferroelectric and the oxide remains poorly known, which limits our understanding of such switching effects. Here, we use a first-of-a-kind combination of scanning transmission electron microscopy with electron energy loss spectroscopy, near-total-reflection hard X-ray photoemission spectroscopy, and ab initio theory to address this issue. We achieve a direct, quantitative, atomic-scale characterization of the polarization-induced charge density changes at the interface between the ferroelectric BiFeO3 and the doped Mott insulator Ca1-xCexMnO3, thus providing insight on how interface-engineering can enhance these switching effects.
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| 12. |
- Marinova, Maya, et al.
(författare)
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Structural and Optical Investigation of VLS Grown (111) 3C-SiC Layers on 6H-SiC Substrates in Sn-Based Melts
- 2011
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Ingår i: Silicon Carbide and Related Materials 2010. ; , s. 165-168
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Konferensbidrag (refereegranskat)abstract
- The current communication focuses on the investigation of 3C-SiC layers grown by the Vapour-Liquid-Solid mechanism on on-axis Si-face 6H-SiC substrates in SiSn melts with different compositions and at different growth temperatures. The layers are studied by Transmission Electron Microscopy and Low Temperature Photoluminescence. It was found that for melts with Sn concentration higher than 60 at% large Sn-related precipitates are formed. The depth distribution of the Sn precipitates strongly depends not only on the melt composition but also on the growth temperature. Their formation strongly influences the stacking fault density and the dopant incorporation in the layers. Lower Sn concentrations combined with higher growth temperatures should result in 3C-SiC layer with enhanced structural quality.
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| 13. |
- Robert, T., et al.
(författare)
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6H-type zigzag faults in low-doped 4H-SiC epitaxial layers.
- 2010
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Ingår i: Mat. Sci. Forum, Vols. 645-648. - 0878492798 - 9780878492794 ; , s. 347-350
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Konferensbidrag (refereegranskat)abstract
- A new type of 6H zigzag faults has been identified from high resolution transmission electron microscopy (HRTEM) measurements performed on low-doped 4H-SiC homoepitaxial layer grown on off-axis substrates in a hot-wall CVD reactor. They are made of half unit cells of 6H with corresponding low temperature photoluminescence (LTPL) response ranging from about 3 eV to 2.5 eV at liquid helium temperature.
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| 14. |
- Singh, I N, et al.
(författare)
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Dynorphin A (1-17) induces apoptosis in striatal neurons in vitro through alpha-amino-3-hydroxy-5-methylisoxazole-4-propionate/kainate receptor-mediated cytochrome c release and caspase-3 activation.
- 2003
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Ingår i: Neuroscience. - : Elsevier BV. - 0306-4522 .- 1873-7544. ; 122:4
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Tidskriftsartikel (refereegranskat)abstract
- Dynorphin A (1-17), an endogenous opioid neuropeptide, can have pathophysiological consequences at high concentrations through actions involving glutamate receptors. Despite evidence of excitotoxicity, the basic mechanisms underlying dynorphin-induced cell death have not been explored. To address this question, we examined the role of caspase-dependent apoptotic events in mediating dynorphin A (1-17) toxicity in embryonic mouse striatal neuron cultures. In addition, the role of opioid and/or glutamate receptors were assessed pharmacologically using dizocilpine maleate (MK(+)801), a non-equilibrium N-methyl-D-aspartate (NMDA) antagonist; 6-cyano-7-nitroquinoxaline-2,3-dione, a competitive alpha-amino-3-hydroxy-5-methylisoxazole-4-propionate (AMPA)/kainate antagonist; or (-)-naloxone, a general opioid antagonist. The results show that dynorphin A (1-17) (>or=10 nM) caused concentration-dependent increases in caspase-3 activity that were accompanied by mitochondrial release of cytochrome c and the subsequent death of cultured mouse striatal neurons. Moreover, dynorphin A-induced neurotoxicity and caspase-3 activation were significantly attenuated by the cell permeable caspase inhibitor, caspase-3 inhibitor-II (z-DEVD-FMK), further suggesting an apoptotic cascade involving caspase-3. AMPA/kainate receptor blockade significantly attenuated dynorphin A-induced cytochrome c release and/or caspase-3 activity, while NMDA or opioid receptor blockade typically failed to prevent the apoptotic response. Last, dynorphin-induced caspase-3 activation was mimicked by the ampakine CX546 [1-(1,4-benzodioxan-6-ylcarbonyl)piperidine], which suggests that the activation of AMPA receptor subunits may be sufficient to mediate toxicity in striatal neurons. These findings provide novel evidence that dynorphin-induced striatal neurotoxicity is mediated by a caspase-dependent apoptotic mechanism that largely involves AMPA/kainate receptors.
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| 15. |
- Toneva, A, et al.
(författare)
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FeSi2 thin films investigated by X-ray photoelectron and infrared spectroscopy
- 2000
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Ingår i: Vacuum. - 0042-207X .- 1879-2715. ; 58:2-3, s. 420-427
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Tidskriftsartikel (refereegranskat)abstract
- Thin films of iron silicide prepared by ion-beam and by electron-beam evaporation of iron on silicon substrates and following rapid thermal processing are investigated. Low-energy argon ion sputtering of surface layer is used for depth profile XPS analysis. The Si2p, Fe2p3/2, O1s and C1s electron spectra are recorded. On the basis of established binding energies and infrared transmission spectra (200–600 cm−1) conclusion are made about the effect of the annealing temperature and duration on the chemical bond type and phase composition of the iron silicide films.
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| 16. |
- Vasiliauskas, Remigijus, et al.
(författare)
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Effect of initial substrate conditions on growth of cubic silicon carbide
- 2011
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Ingår i: Journal of Crystal Growth. - : Elsevier Science B.V., Amsterdam.. - 0022-0248 .- 1873-5002. ; 324:1, s. 7-14
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Tidskriftsartikel (refereegranskat)abstract
- In order to analyze the epitaxial growth of cubic silicon carbide by sublimation epitaxy on different substrates, four different 6H-SiC substrate preparations were used: (i) as-received, (ii) re-polished, (iii) annealed and covered by silicon layer and (iv) with (1 1 1) 3C-SiC buffer layer. Almost 100% coverage and low twin density was achieved when grown on the buffer layer. The XRD and TEM characterizations show better material quality when the layer is grown directly on 6H-SiC substrates. Background doping evaluated by LTPL is in the range of 10(16) cm(-3) for N and 10(16) cm(-3) for Al in all grown layers.
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| 17. |
- Vasiliauskas, Remigijus, et al.
(författare)
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Polytype transformation and structural characteristics of 3C-SiC on 6H-SiC substrates
- 2014
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Ingår i: Journal of Crystal Growth. - : Elsevier BV. - 0022-0248 .- 1873-5002. ; 395, s. 109-115
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Tidskriftsartikel (refereegranskat)abstract
- The 3C-SiC (111) was grown on on-axis 6H-SiC substrates in a temperature interval ranging from 1675oC where 3C-SiC nucleated, to 1825oC where coverage of the substrate by 3C-SiC was nearly 100%. The 6H- to 3C-SiC transformation was not abrupt and two different transitions could be observed. The first one occurs before or during 3C-SiC nucleation and consists of 6H-, 3C-, 15R-SiC and other unresolved stacking sequences. The second one appears due to 6H-SiC and 3C-SiC competition during the growth and results in non flat needle-like interface. A proposed model elucidates connection between four-fold twins nucleating at the 6H-/3C-SiC interface and the formation of depressions at the surface of the 3C-SiC layer.
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| 18. |
- Vasiliauskas, Remigijus, et al.
(författare)
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Sublimation Growth and Structural Characterization of 3C-SiC on Hexagonal and Cubic SiC Seeds
- 2010
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Ingår i: Materials Science Forum, Vols. 645-648. - : Transtec Publications; 1999. ; , s. 175-178
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Konferensbidrag (refereegranskat)abstract
- Epitaxial growth of cubic silicon carbide on 6H-SiC substrates, and 6H-SiC substrates with (111) 3C-SiC buffer layer, deposited by vapour liquid solid mechanism, was compared. The morphological details of the grown layers were studied by optical microscopy and their microstructure by transmission electron microscopy. The influence of the substrate on the nucleation of 3C-SiC, the initial homoepitaxial 6H-SiC nucleation before 3C-SiC as well as the formation of defects, are discussed.
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