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| 2. |
- Vogel, G. F., et al.
(författare)
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Genotypic and phenotypic spectrum of infantile liver failure due to pathogenic TRMU variants
- 2023
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Ingår i: Genetics in Medicine. - : Elsevier BV. - 1098-3600. ; 25:6
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: This study aimed to define the genotypic and phenotypic spectrum of reversible acute liver failure (ALF) of infancy resulting from biallelic pathogenic TRMU variants and determine the role of cysteine supplementation in its treatment. Methods: Individuals with biallelic (likely) pathogenic variants in TRMU were studied within an international retrospective collection of de-identified patient data. Results: In 62 individuals, including 30 previously unreported cases, we described 47 (likely) pathogenic TRMU variants, of which 17 were novel, and 1 intragenic deletion. Of these 62 individuals, 42 were alive at a median age of 6.8 (0.6-22) years after a median follow-up of 3.6 (0.1-22) years. The most frequent finding, occurring in all but 2 individuals, was liver involvement. ALF occurred only in the first year of life and was reported in 43 of 62 individuals; 11 of whom received liver transplantation. Loss-of-function TRMU variants were associated with poor survival. Supplementation with at least 1 cysteine source, typically N-acetylcysteine, improved survival significantly. Neurodevelopmental delay was observed in 11 individuals and persisted in 4 of the survivors, but we were unable to determine whether this was a primary or a secondary consequence of TRMU deficiency. Conclusion: In most patients, TRMU-associated ALF was a transient, reversible disease and cysteine supplementation improved survival.
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| 4. |
- Kramer, K. P., et al.
(författare)
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Band structure of overdoped cuprate superconductors: Density functional theory matching experiments
- 2019
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Ingår i: Physical Review B. - 2469-9969 .- 2469-9950. ; 99:22
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Tidskriftsartikel (refereegranskat)abstract
- A comprehensive angle-resolved photoemission spectroscopy study of the band structure in singlelayer cuprates is presented with the aim of uncovering universal trends across different materials. Five different hole-and electron-overdoped cuprate superconductors (La1.59Eu0.2Sr0.21CuO4, La1.77Sr0.23CuO4, Bi1.74Pb0.38Sr1.88CuO6+delta, Tl2Ba2CuO6+delta, and Pr1.15La0.7Ce0.15CuO4) have been studied with special focus on the bands with a predominately d-orbital character. Using a light polarization analysis, the e(g) and t(2g) bands are identified across these materials. A clear correlation between the d(3z2-r2) band energy and the apical oxygen distance d(A) is demonstrated. Moreover, the compound dependence of the d(x2-y2) band bottom and the t(2g) band top is revealed. A direct comparison to density functional theory (DFT) calculations employing hybrid exchange-correlation functionals demonstrates excellent agreement. We thus conclude that the DFT methodology can be used to describe the global band structure of overdoped single-layer cuprates on both the hole-and electron-doped side.
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| 5. |
- Schroter, D, et al.
(författare)
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Ecosystem service supply and vulnerability to global change in Europe
- 2005
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Ingår i: Science. - : American Association for the Advancement of Science (AAAS). - 1095-9203 .- 0036-8075. ; 310:5752, s. 1333-1337
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Tidskriftsartikel (refereegranskat)abstract
- Global change will alter the supply of ecosystem services that are vital for human well-being. To investigate ecosystem service supply during the 21st century, we used a range of ecosystem models and scenarios of climate and land-use change to conduct a Europe-wide assessment. Large changes in climate and land use typically resulted in large changes in ecosystem service supply. Some of these trends may be positive (for example, increases in forest area and productivity) or offer opportunities (for example, "surplus land" for agricultural extensification and bioenergy production). However, many changes increase vulnerability as a result of a decreasing supply of ecosystem services (for example, declining soil fertility, declining water availability, increasing risk of forest fires), especially in the Mediterranean and mountain regions.
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| 6. |
- Engelmann, U, et al.
(författare)
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Experiences with the German teleradiology system MEDICUS
- 1997
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Ingår i: COMPUTER METHODS AND PROGRAMS IN BIOMEDICINE. - 0169-2607. ; 54:1-2, s. 131-139
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- This paper introduces the teleradiology system, MEDICUS, which has been developed at the Deutsches Krebsforschungszentrum (German Cancer Research Center) in Heidelberg, Germany. The system is designed to work on ISDN lines as well as in a local area netwo
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| 7. |
- Bauer, Maris, et al.
(författare)
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The potential for sensitivity enhancement by the thermoelectric effect in carbon-nanotube and graphene Tera-FETs
- 2015
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Ingår i: Journal of Physics: Conference Series. - : IOP Publishing. - 1742-6588 .- 1742-6596. ; 647:1, s. UNSP 012004-
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Konferensbidrag (refereegranskat)abstract
- We report on terahertz (THz) measurements with graphene field-effect transistors with integrated antennas (Tera-FETs) lay special emphasis on thermoelectric contributions to the detected THz photoresponse. Graphene Tera-FETs with integrated broad-band bow-tie antennas were fabricated in a CVD-based growth process and were successfully applied for detection at 600 GHz with optical NEPs down to 515 pW/Hz^1/2. While rectification of THz radiation by (distributed) resistive mixing of charge-density waves induced in the gated transistor channel region is well known, significant additional contributions to the detected signal have experimentally been observed and hot-carrier thermoelectric effects have been identified as a possible origin of these signals. We also observe similar signal contributions in carbonnanotube transistors.
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| 8. |
- Sakalas, P., et al.
(författare)
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Harmonic Distortion Analysis of InP HBTs with 650 GHz f(max) for High Data Rate Communication Systems
- 2017
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Ingår i: IEEE Compound Semiconductor Integrated Circuit Symposium Technical Digest. - 1550-8781. - 9781509060696
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Konferensbidrag (refereegranskat)abstract
- High frequency (h.f.) harmonic distortion (HD) of advanced InP heterojunction bipolar transistors (HBTs) with various emitter widths was investigated. Geometry scalable parameters for the compact model (CM) HICUM/L2 v. 2.34, featuring a two-region base-collector capacitance formulation, were extracted from temperature dependent DC and AC measurements of HBTs and from the special test structures. Single tone harmonic distortion and active two tone load pull measurements were carried out for different emitter area devices. The compact model was used for data analysis.
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| 9. |
- Sakalas, P., et al.
(författare)
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Microwave Noise Analysis in InP and GaAs HBTs
- 2016
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Ingår i: Technical Digest - IEEE Compound Semiconductor Integrated Circuit Symposium, CSIC. - 1550-8781. - 9781509016082 ; 2016-November, s. Art. no 7751054-
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Konferensbidrag (refereegranskat)abstract
- High frequency (h.f.) noise characteristics of advanced InP and GaAs HBTs were measured and simulated. The compact model (CM) HICUM/L2 v2.34 was used for the DC, AC and noise simulation as well as for the noise analysis. Geometry scalable model parameters for InP HBTs with the different emitter widths and lengths were extracted from temperature dependent DC and AC measurements on HBTs and special test structures. The CM is in good agreement with measured data. Non-equilibrium electron transport was found to shape fT and fmax for GaAs HBTs. For both HBT types, based on the noise source decomposition, an analysis of the influence of the different noise sources on the minimum noise figure (NFmin) was performed at different base-collector biases VBC. It was found that noise due to intervalley transfer related electron scattering has negligible impact on NFmin for both InP and GaAs HBTs. H.f. noise reduction as a result of Coulomb current blocking in GaAs HBTs was confirmed. Shot noise correlation was investigated in GaAs HBTs with different base layer thickness (wB) and base doping for the optimal h.f. noise behavior.
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| 10. |
- Sakalas, P., et al.
(författare)
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mm-Wave noise modeling in advanced SiGe and InP HBTs
- 2015
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Ingår i: Journal of Computational Electronics. - : Springer Science and Business Media LLC. - 1569-8025 .- 1572-8137. ; 14:1, s. 62-71
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Tidskriftsartikel (refereegranskat)abstract
- DC, RF and noise characteristics of advanced InP/InGaAs and Si/SiGe heterojunction bipolar transistors (HBTs) were measured and modeled in a broad frequency range. Equations for systematically modeled correlated noise in bipolar transistors and their implementation in the compact models HICUM/L0 and L2 are proposed. The models are verified for advanced SiGe HBTs up to 300 GHz by hydrodynamic device simulation and by results from the Boltzmann transport equation. The verified model was used for analyzing the noise of advanced InP/InGaAs and Si/SiGe HBTs. Compared to Si/SiGe HBTs a higher noise at lower frequencies was observed in InP/InGaAs HBTs due to a higher base recombination current. InP HBTs shows a good noise performance beyond 100 GHz and due to their better product can compete with advanced Si/SiGe HBTs for LNA design at mm-wave frequencies.
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