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- Niemi, MEK, et al.
(författare)
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- 2021
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swepub:Mat__t
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| 4. |
- Hikmat, O., et al.
(författare)
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Expanding the phenotypic spectrum of BCS1L-related mitochondrial disease
- 2021
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Ingår i: Annals of Clinical and Translational Neurology. - : Wiley. - 2328-9503. ; 8:11, s. 2155-2165
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To delineate the full phenotypic spectrum of BCS1L-related disease, provide better understanding of the genotype-phenotype correlations and identify reliable prognostic disease markers. Methods: We performed a retrospective multinational cohort study of previously unpublished patients followed in 15 centres from 10 countries. Patients with confirmed biallelic pathogenic BCS1L variants were considered eligible. Clinical, laboratory, neuroimaging and genetic data were analysed. Patients were stratified into different groups based on the age of disease onset, whether homozygous or compound heterozygous for the c.232A>G (p.Ser78Gly) variant, and those with other pathogenic BCS1L variants. Results: Thirty-three patients were included. We found that growth failure, lactic acidosis, tubulopathy, hepatopathy and early death were more frequent in those with disease onset within the first month of life. In those with onset after 1 month, neurological features including movement disorders and seizures were more frequent. Novel phenotypes, particularly involving movement disorder, were identified in this group. The presence of the c.232A>G (p.Ser78Gly) variant was associated with significantly worse survival and exclusively found in those with disease onset within the first month of life, whilst other pathogenic BCS1L variants were more frequent in those with later symptom onset. Interpretation: The phenotypic spectrum of BCS1L-related disease comprises a continuum of clinical features rather than a set of separate syndromic clinical identities. Age of onset defines BCS1L-related disease clinically and early presentation is associated with poor prognosis. Genotype correlates with phenotype in the presence of the c.232A>G (p.Ser78Gly) variant.
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The Seventeenth Data Release of the Sloan Digital Sky Surveys : Complete Release of MaNGA, MaStar, and APOGEE-2 Data
- 2022
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Ingår i: Astrophysical Journal Supplement Series. - : Institute of Physics (IOP). - 0067-0049 .- 1538-4365. ; 259:2
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Tidskriftsartikel (refereegranskat)abstract
- This paper documents the seventeenth data release (DR17) from the Sloan Digital Sky Surveys; the fifth and final release from the fourth phase (SDSS-IV). DR17 contains the complete release of the Mapping Nearby Galaxies at Apache Point Observatory (MaNGA) survey, which reached its goal of surveying over 10,000 nearby galaxies. The complete release of the MaNGA Stellar Library accompanies this data, providing observations of almost 30,000 stars through the MaNGA instrument during bright time. DR17 also contains the complete release of the Apache Point Observatory Galactic Evolution Experiment 2 survey that publicly releases infrared spectra of over 650,000 stars. The main sample from the Extended Baryon Oscillation Spectroscopic Survey (eBOSS), as well as the subsurvey Time Domain Spectroscopic Survey data were fully released in DR16. New single-fiber optical spectroscopy released in DR17 is from the SPectroscipic IDentification of ERosita Survey subsurvey and the eBOSS-RM program. Along with the primary data sets, DR17 includes 25 new or updated value-added catalogs. This paper concludes the release of SDSS-IV survey data. SDSS continues into its fifth phase with observations already underway for the Milky Way Mapper, Local Volume Mapper, and Black Hole Mapper surveys.
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| 6. |
- Grawe, H., et al.
(författare)
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The (6+) isomer in 102Sn revisited : Neutron and proton effective charges close to the double shell closure
- 2021
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Ingår i: Physics Letters B. - : Elsevier. - 0370-2693 .- 1873-2445. ; 820
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Tidskriftsartikel (refereegranskat)abstract
- In a high-energy fragmentation experiment at GSI an Iπ = (6+) isomer and its γ-decay are identified in 102Sn, the two-neutron neighbour of the doubly-magic 100Sn. Its half-life is measured to be T1/2 = 367(11) ns. The possible existence of further isomers is discussed in the framework of large-scale shell model (LSSM) calculations including up to five particle-hole excitations of the 100Sn core. From the precise B(E2; 6+ → 4+) strength and the recently remeasured value for B(E2; 8+ → 6+) in the two-proton hole neighbour 98Cd effective E2 polarization charges for protons and neutrons were inferred including LSSM corrections within the full N=4 0hω space. The results are discussed in comparison to predicted and empirically determined effective operators.
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| 11. |
- Parikh, V. K., et al.
(författare)
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Current status on manufacturing routes to produce metal matrix composites : State-of-the-art
- 2023
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Ingår i: Heliyon. - : Elsevier BV. - 2405-8440. ; 9:2
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Tidskriftsartikel (refereegranskat)abstract
- Owing to its excellent properties, Metal Matrix Composites (MMC) has gained popularity and finds application in aerospace, aircraft, shipbuilding, biomedical, biodegradable implant materials and many more. To serve the industrial needs, the manufactured MMC should have homogenous distribution along with minimum agglomeration of reinforcement particles, defect-free microstructure, superior mechanical, tribological and corrosive properties. The techniques implemented to manufacture MMC highly dominate the aforementioned characteristics. According to the physical state of the matrix, the techniques implemented for manufacturing MMC can be classified under two categories i.e. solid state processing and liquid state process. The present article attempts to review the current status of different manufacturing techniques covered under these two categories. The article elaborates on the working principles of state-of-the-art manufacturing techniques, the effect of dominating process parameters and the resulting characteristic of composites. Apart from this, the article does provide data regarding the range of dominating process parameters and resulting mechanical properties of different grades of manufactured MMC. Using this data along with the comparative study, various industries and academicians will be able to select the appropriate techniques for manufacturing MMC.
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| 12. |
- Russell, T. D., et al.
(författare)
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Rapid compact jet quenching in the Galactic black hole candidate X-ray binary MAXI J1535-571
- 2020
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Ingår i: Monthly Notices of the Royal Astronomical Society. - : Oxford University Press (OUP). - 0035-8711 .- 1365-2966. ; 498:4, s. 5772-5785
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Tidskriftsartikel (refereegranskat)abstract
- We present results from six epochs of quasi-simultaneous radio, (sub-)millimetre, infrared, optical, and X-ray observations of the black hole X-ray binary MAXI J1535-571. These observations show that as the source transitioned through the hard-intermediate X-ray state towards the soft-intermediate X-ray state, the jet underwent dramatic and rapid changes. We observed the frequency of the jet spectral break, which corresponds to the most compact region in the jet where particle acceleration begins (higher frequencies indicate closer to the black hole), evolves from the infrared band into the radio band (decreasing by approximate to 3 orders of magnitude) in less than a day. During one observational epoch, we found evidence of the jet spectral break evolving in frequency through the radio band. Estimating the magnetic field and size of the particle acceleration region shows that the rapid fading of the high-energy jet emission was not consistent with radiative cooling; instead, the particle acceleration region seems to be moving away from the black hole on approximately dynamical time-scales. This result suggests that the compact jet quenching is not caused by local changes to the particle acceleration, rather we are observing the acceleration region of the jet travelling away from the black hole with the jet flow. Spectral analysis of the X-ray emission shows a gradual softening in the few days before the dramatic jet changes, followed by a more rapid softening similar to 1-2 d after the onset of the jet quenching.
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