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Sökning: WFRF:(Henrich G)

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1.
  • Mercuri, E., et al. (författare)
  • Safety and effectiveness of ataluren: comparison of results from the STRIDE Registry and CINRG DMD Natural History Study
  • 2020
  • Ingår i: Journal of Comparative Effectiveness Research. - : Becaris Publishing Limited. - 2042-6305 .- 2042-6313. ; 9:5, s. 341-360
  • Tidskriftsartikel (refereegranskat)abstract
    • Aim: Strategic Targeting of Registries and International Database of Excellence (STRIDE) is an ongoing, multicenter registry providing real-world evidence regarding ataluren use in patients with nonsense mutation Duchenne muscular dystrophy (nmDMD). We examined the effectiveness of ataluren + standard of care (SoC) in the registry versus SoC alone in the Cooperative International Neuromuscular Research Group (CINRG) Duchenne Natural History Study (DNHS), DMD genotype-phenotype/-ataluren benefit correlations and ataluren safety. Patients & methods: Propensity score matching was performed to identify STRIDE and CINRG DNHS patients who were comparable in established disease progression predictors (registry cut-off date, 9 July 2018). Results & conclusion: Kaplan-Meier analyses demonstrated that ataluren + SoC significantly delayed age at loss of ambulation and age at worsening performance in timed function tests versus SoC alone (p <= 0.05). There were no DMD genotype-phenotype/ataluren benefit correlations. Ataluren was well tolerated. These results indicate that ataluren + SoC delays functional milestones of DMD progression in patients with nmDMD in routine clinical practice. ClinicalTrials.gov identifier: NCT02369731. ClinicalTrials.gov identifier: NCT02369731.
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2.
  • Henrich, C., et al. (författare)
  • Coulomb excitation of 142Xe
  • 2018
  • Ingår i: Acta Physica Polonica B. - 0587-4254. ; 49:3, s. 529-533
  • Tidskriftsartikel (refereegranskat)abstract
    • The even-even nucleus 142Xe lies north-east of the doubly magic 132Sn on the neutron-rich side of the nuclear chart. In order to gain further information on the octupole collectivity and the evolution of quadrupole collectivity in this region, a "safe" Coulomb excitation experiment was carried out at the new HIE-ISOLDE facility (CERN) at the end of 2016. As the gamma-ray detector the Miniball spectrometer was used. Beam and target nuclei were detected using C-REX, i.e. an array of segmented Si detectors, covering forward as well as backward angles in the laboratory frame.
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3.
  • Jentschel, M., et al. (författare)
  • EXILL - a high-efficiency, high-resolution setup for gamma-spectroscopy at an intense cold neutron beam facility
  • 2017
  • Ingår i: Journal of Instrumentation. - : IOP PUBLISHING LTD. - 1748-0221. ; 12
  • Tidskriftsartikel (refereegranskat)abstract
    • In the EXILL campaign a highly efficient array of high purity germanium (HPGe) detectors was operated at the cold neutron beam facility PF1B of the Institut Laue-Langevin (ILL) to carry out nuclear structure studies, via measurements of gamma-rays following neutron-induced capture and fission reactions. The setup consisted of a collimation system producing a pencil beam with a thermal capture equivalent flux of about 10(8) ns(-1)cm(2) at the target position and negligible neutron halo. The targetwas surrounded by an array of eight to ten anti-Compton shielded EXOGAMClover detectors, four to six anti-Compton shielded large coaxial GASP detectors and two standard Clover detectors. For a part of the campaign the array was combined with 16 LaBr3:(Ce) detectors from the FATIMA collaboration. The detectorswere arranged in an array of rhombicuboctahedron geometry, providing the possibility to carry out very precise angular correlation and directional-polarization correlation measurements. The triggerless acquisition system allowed a signal collection rate of up to 6 x 10(5) Hz. The data allowed to set multi-fold coincidences to obtain decay schemes and in combination with the FATIMA array of LaBr3:(Ce) detectors to analyze half-lives of excited levels in the pico-to microsecond range. Precise energy and efficiency calibrations of EXILL were performed using standard calibration sources of Ba-133, Co-60 and Eu-152 as well as data from the reactions Al-27(n, gamma)Al-28 and Cl-35(n,gamma)Cl-36 in the energy range from 30 keV up to 10MeV.
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4.
  • Goldkuhle, A., et al. (författare)
  • Lifetime measurements in Ti-52,Ti-54 to study shell evolution toward N=32
  • 2019
  • Ingår i: Physical Review C. - : American Physical Society. - 2469-9985 .- 2469-9993. ; 100:5
  • Tidskriftsartikel (refereegranskat)abstract
    • Lifetimes of the excited states in the neutron-rich Ti-52,Ti-54 nuclei, produced in a multinucleon-transfer reaction, were measured by employing the Cologne plunger device and the recoil-distance Doppler-shift method. The experiment was performed at the Grand Accelerateur National d'Ions Lourds facility by using the Advanced Gamma Tracking Array for the gamma-ray detection, coupled to the large-acceptance variable mode spectrometer for an event-by-event particle identification. A comparison between the transition probabilities obtained from the measured lifetimes of the 2(1)(+) to 8(1)(+) yrast states in Ti-52,Ti-54 and that from the shell-model calculations based on the well-established GXPF1A, GXPF1B, and KB3G fp shell interactions support the N = 32 subshell closure. The B(E2) values for Ti-52 determined in this work are in disagreement with the known data, but are consistent with the predictions of the shell-model calculations and reduce the previously observed pronounced staggering across the even-even titanium isotopes.
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5.
  • Gjestvang, D., et al. (författare)
  • Examination of how properties of a fissioning system impact isomeric yield ratios of the fragments
  • 2023
  • Ingår i: Physical Review C. - : American Physical Society. - 2469-9985 .- 2469-9993. ; 108:6
  • Tidskriftsartikel (refereegranskat)abstract
    • The population of isomeric states in the prompt decay of fission fragments-so-called isomeric yield ratios (IYRs)-is known to be sensitive to the angular momentum J that the fragment emerged with, and may therefore contain valuable information on the mechanism behind the fission process. In this work, we investigate how changes in the fissioning system impact the measured IYRs of fission fragments to learn more about what parameters affect angular momentum generation. To enable this, a new technique for measuring IYRs is first demonstrated. It is based on the time of arrival of discrete gamma rays, and has the advantage that it enables the study of the IYR as a function of properties of the partner nucleus. This technique is used to extract the IYR of 134Te, strongly populated in actinide fission, from the three different fissioning systems: 232Th(n, f), 238U(n, f), at two different neutron energies, as well as 252Cf(sf). The impacts of changing the fissioning system, the compound nuclear excitation energy, the minimum J of the binary partner, and the number of neutrons emitted on the IYR of 134Te are determined. The decay code TALYS is used in combination with the fission simulation code FREYA to calculate the primary fragment angular momentum from the IYR. We find that the IYR of 134Te has a slope of 0.004 +/- 0.002 with increase in compound nucleus (CN) mass. When investigating the impact on the IYR of increased CN excitation energy, we find no change with an energy increase similar to the difference between thermal and fast fission. By varying the mass of the partner fragment emerging with 134Te, it is revealed that the IYR of 134Te is independent of the total amount of prompt neutrons emitted from the fragment pair. This indicates that neutrons carry minimal angular momentum away from the fission fragments. Comparisons with the FREYA+TALYS simulations reveal that the average angular momentum in 134Te following 238U(n, f) is 6.0 h over bar . This is not consistent with the value deduced from recent CGMF calculations. Finally, the IYR sensitivity to the angular momentum of the primary fragment is discussed. These results are not only important to help understanding the underlying mechanism in nuclear fission, but can also be used to constrain and benchmark fission models, and are relevant to the gamma -ray heating problem of reactors.
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6.
  • Rosiak, D., et al. (författare)
  • Enhanced Quadrupole and Octupole Strength in Doubly Magic Sn 132
  • 2018
  • Ingår i: Physical Review Letters. - 0031-9007. ; 121:25
  • Tidskriftsartikel (refereegranskat)abstract
    • The first 2+ and 3- states of the doubly magic nucleus Sn132 are populated via safe Coulomb excitation employing the recently commissioned HIE-ISOLDE accelerator at CERN in conjunction with the highly efficient MINIBALL array. The Sn132 ions are accelerated to an energy of 5.49 MeV/nucleon and impinged on a Pb206 target. Deexciting γ rays from the low-lying excited states of the target and the projectile are recorded in coincidence with scattered particles. The reduced transition strengths are determined for the transitions 0g.s.+→21+, 0g.s.+→31-, and 21+→31- in Sn132. The results on these states provide crucial information on cross-shell configurations which are determined within large-scale shell-model and Monte Carlo shell-model calculations as well as from random-phase approximation and relativistic random-phase approximation. The locally enhanced B(E2;0g.s.+→21+) strength is consistent with the microscopic description of the structure of the respective states within all theoretical approaches. The presented results of experiment and theory can be considered to be the first direct verification of the sphericity and double magicity of Sn132.
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8.
  • Butler, P. A., et al. (författare)
  • The observation of vibrating pear-shapes in radon nuclei
  • 2019
  • Ingår i: Nature Communications. - : Springer Science and Business Media LLC. - 2041-1723. ; 10:1
  • Tidskriftsartikel (refereegranskat)abstract
    • There is a large body of evidence that atomic nuclei can undergo octupole distortion and assume the shape of a pear. This phenomenon is important for measurements of electric-dipole moments of atoms, which would indicate CP violation and hence probe physics beyond the Standard Model of particle physics. Isotopes of both radon and radium have been identified as candidates for such measurements. Here, we observed the low-lying quantum states in 224Rn and 226Rn by accelerating beams of these radioactive nuclei. We show that radon isotopes undergo octupole vibrations but do not possess static pear-shapes in their ground states. We conclude that radon atoms provide less favourable conditions for the enhancement of a measurable atomic electric-dipole moment.
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9.
  • Klug, G., et al. (författare)
  • The therapist variable : Is it still an unknown factor? [Die Therapeutenvariable
  • 2008
  • Ingår i: Psychotherapeut (Berlin). - : Springer Science and Business Media LLC. - 0935-6185 .- 1432-2080. ; 53:2, s. 83-91
  • Tidskriftsartikel (refereegranskat)abstract
    • The objective of this study was to investigate the therapeutic attitude as a self-contained dimension of the therapeutic process. The psychotherapists' beliefs and attitudes in therapeutic matters are measured according to the Therapeutic Attitude Scales (TASC) of the Therapeutic Attitude Questionnaire (ThAt) and were applied to investigate the therapist variable. The investigation concentrated on the therapist's belief in the curative value of a number of ingredients of psychotherapy, on the therapist's individual style of conducting psychotherapy and on basic assumptions about the nature of psychotherapy and the nature of the human mind. A total of 451 psychotherapists (81 psychodynamic psychotherapists, 208 psychoanalytic psychotherapists and 162 behaviour therapists) were investigated. There were highly significant differences between the three groups of therapists, a finding which is in line with the basic theory. Adaptation and insight of the assumed curative factors as well as supportiveness and neutrality of the technique factors, differentiate the groups highly significantly. The multivariate analysis underlines the importance of adaptation and insight to differentiate between all groups. The results by and large confirm the general assumptions held on the differences between the groups. Thus the ThAt appears to be a valid instrument to grasp more subtle features of the therapist variable that may influence the outcome of psychotherapy. © 2008 Springer Medizin Verlag.
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10.
  • Lee, V., et al. (författare)
  • Imaging high energy photons with PILATUS II at the tagged photon beam at MAX-lab
  • 2009
  • Ingår i: Nuclear Instruments & Methods in Physics Research. Section A: Accelerators, Spectrometers, Detectors, and Associated Equipment. - : Elsevier BV. - 0167-5087 .- 0168-9002. ; 603:3, s. 379-383
  • Tidskriftsartikel (refereegranskat)abstract
    • In photonuclear experiments precise location of the photon beam relative to the experimental sample is critical. Previously used techniques such as using photographic film to identify the position, intensity and centroid of the beam is time-consuming and a faster method is required. PILATUS is a single-photon-counting pixel detector developed at the Paul Scherrer Institute (PSI), Switzerland. It is a silicon-based, two-dimensional detector with a large dynamic range and zero readout noise. Designed as an X-ray detector, its optimal quantum efficiency is between 3 and 30 keV. This paper reports measurements carried out at the MAX-lab tagged photon facility in Lund, Sweden. The beam endpoint energy of approximately 200 MeV is far above the designed optimal energy detection range of PILATUS, and provides a critical test of the use of PILATUS under high energy conditions. The detector was placed in the photon beam and images were taken both downstream of other experiments, and in close range of a 19 mm collimator. The successful measurements demonstrate the versatility and robustness of the detector and provide an effective way of quickly and accurately monitoring beam position and profile in real time. (C) 2009 Elsevier B.V. All rights reserved.
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12.
  • Al Jebali, Ramsey, et al. (författare)
  • Recent Developments SoNDe High-Flux Detector Project
  • 2018
  • Ingår i: Recent Developments SoNDe High-Flux Detector Project.
  • Konferensbidrag (refereegranskat)abstract
    • New high-flux and high-brilliance neutron sources demand a higher count-rate capability in neutron detectors. In order to achieve that goal, the Solid-State Neutron Detector (SoNDe) project is developing a scintillation-based neutron detector. It will be capable of fully exploiting the available flux at small-angle neutron scattering (SANS) instruments at high brilliance sources, such as SKADI at the European Spallation Source (ESS). The read-out of the scintillator is based on a pixelized multi-anode PMT (MaPMT), where each pixel is treated separately. In addition to enabling higher achievable count-rates, one of the design goals was to develop a modular and scalable solution that can also be used in other instruments or even contexts, such as for laboratory setups. This has been achieved by combining the complete read-out electronics along with the MaPMT into modules that can be controlled and read-out individually via a network without additional any infrastructure. An overview of the present state of development and current test results is presented, highlighting the results of previously published project reports.
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13.
  • Butler, P. A., et al. (författare)
  • Evolution of Octupole Deformation in Radium Nuclei from Coulomb Excitation of Radioactive ^{222}Ra and ^{228}Ra Beams
  • 2020
  • Ingår i: Physical Review Letters. - 1079-7114. ; 124:4
  • Tidskriftsartikel (refereegranskat)abstract
    • There is sparse direct experimental evidence that atomic nuclei can exhibit stable "pear" shapes arising from strong octupole correlations. In order to investigate the nature of octupole collectivity in radium isotopes, electric octupole (E3) matrix elements have been determined for transitions in ^{222,228}Ra nuclei using the method of sub-barrier, multistep Coulomb excitation. Beams of the radioactive radium isotopes were provided by the HIE-ISOLDE facility at CERN. The observed pattern of E3 matrix elements for different nuclear transitions is explained by describing ^{222}Ra as pear shaped with stable octupole deformation, while ^{228}Ra behaves like an octupole vibrator.
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14.
  • Chung, J S, et al. (författare)
  • A remarkable, precisely timed release of hyperglycemic hormone from endocrine cells in the gut is associated with ecdysis in the crab Carcinus maenas
  • 1999
  • Ingår i: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 96:23, s. 13103-7
  • Tidskriftsartikel (refereegranskat)abstract
    • Molting or ecdysis is the most fundamentally important process in arthropod life history, because shedding of the exoskeleton is an absolute prerequisite for growth and metamorphosis. Although the hormonal mechanisms driving ecdysis in insects have been studied extensively, nothing is known about these processes in crustaceans. During late premolt and during ecdysis in the crab Carcinus maenas, we observed a precise and reproducible surge in hemolymph hyperglycemic hormone (CHH) levels, which was over 100-fold greater than levels seen in intermolt animals. The source of this hormone surge was not from the eyestalk neurosecretory tissues but from previously undescribed endocrine cells (paraneurons), in defined areas of the foregut and hindgut. During premolt (the only time when CHH is expressed by these tissues), the gut is the largest endocrine tissue in the crab. The CHH surge, which is a result of an unusual, almost complete discharge of the contents of the gut endocrine cell, regulates water and ion uptake during molting, thus allowing the swelling necessary for successful ecdysis and the subsequent increase in size during postmolt. This study defines an endocrine brain/gut axis in the arthropods. We propose that the ionoregulatory process controlled by CHH may be common to arthropods, in that, for insects, a similar mechanism seems to be involved in antidiuresis. It also seems likely that a cascade of very precisely coordinated release of (neuro) hormones controls ecdysis.
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15.
  • Guerrero Bosagna, Carlos, et al. (författare)
  • DNA methylation variation in the brain of laying hens in relation to differential behavioral patterns
  • 2020
  • Ingår i: Comparative Biochemistry and Physiology - Part D. - : ELSEVIER SCIENCE INC. - 1744-117X .- 1878-0407. ; 35
  • Tidskriftsartikel (refereegranskat)abstract
    • Domesticated animals are unique to investigate the contribution of genetic and non-genetic factors to specific phenotypes. Among non-genetic factors involved in phenotype formation are epigenetic mechanisms. Here we aimed to identify whether relative DNA methylation differences in the nidopallium between groups of individuals are among the non-genetic factors involved in the emergence of differential behavioral patterns in hens. The nidopallium was selected due to its important role in complex cognitive function (i.e., decision making) in birds. Behavioral patterns that spontaneously emerge in hens living in a highly controlled environment were identified with a unique tracking system that recorded their transitions between pen zones. Behavioral activity patterns were characterized through three classification schemes: (i) daily specific features of behavioral routines (Entropy), (ii) daily spatio-temporal activity patterns (Dynamic Time Warping), and (iii) social leading behavior (Leading Index). Unique differentially methylated regions (DMRs) were identified between behavioral patterns emerging within classification schemes, with entropy having the higher number. Functionally, DTW had double the proportion of affected promoters and half of the distal intergenic regions. Pathway enrichment analysis of DMR-associated genes revealed that Entropy relates mainly to cell cycle checkpoints, Leading Index to mitochondrial function, and DTW to gene expression regulation. Our study suggests that different biological functions within neurons (particularly in the nidopallium) could be responsible for the emergence of distinct behavior patterns and that epigenetic variation within brain tissues would be an important factor to explain behavioral variation.
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16.
  • Illana, A., et al. (författare)
  • Coulomb excitation of 74,76Zn
  • 2023
  • Ingår i: Physical Review C. - 2469-9985. ; 108:4
  • Tidskriftsartikel (refereegranskat)abstract
    • The first experiment using radioactive beams post-accelerated by the HIE-ISOLDE facility has enabled to obtain a precise set of B(E2) transition probabilities in neutron-rich 74,76Zn isotopes. The resulting B(E2; 2+1→0+1) values are consistent with those determined in earlier REX-ISOLDE measurements. While the B(E2; 4+1→2+1) transition probability in 76Zn is also in agreement with earlier Coulomb-excitation results, the value obtained for 74Zn is considerably lower. For the first time, a spectroscopic quadrupole moment of the 2+1 state was measured for an exotic nucleus in this mass region. A detailed comparison is presented with large-scale shell-model and Monte Carlo shell-model calculations.
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17.
  • Peluso, Michael J, et al. (författare)
  • Cerebrospinal fluid soluble CD30 elevation despite suppressive antiretroviral therapy in individuals living with HIV-1.
  • 2020
  • Ingår i: Journal of virus eradication. - 2055-6640. ; 6:1, s. 19-26
  • Tidskriftsartikel (refereegranskat)abstract
    • The aim of this study was to assess soluble CD30 (sCD30), a protein that colocalises with HIV-1 RNA and DNA in lymphoid cells and tissues, in cerebrospinal fluid (CSF) as a marker of HIV-1 infection in the central nervous system (CNS).This was a cross-sectional study using archived samples from two clinical cohorts. Soluble CD30 concentrations were measured in paired CSF and plasma from untreated viraemic individuals (n=52), individuals on suppressive antiretroviral therapy (ART) (n=33), HIV-1 controllers (n=10), participants with CSF HIV-1 'escape' (n=11) and controls without HIV-1 infection (n=16). Nonparametric tests were used to compare levels across groups and evaluate correlations with HIV-1 RNA, CSF neurofilament light chain protein (NFL) and neopterin.Compared with controls (median 30ng/mL, interquartile range [IRQ] 23-50), plasma sCD30 levels were elevated in viraemic participants (75ng/mL, 52-116; P<0.001), but not in those on suppressive ART (38ng/mL, 32-62). In contrast, CSF sCD30 levels were elevated in ART-suppressed individuals (34ng/mL, 19-46; P=0.001) and in those with CSF 'escape' (33ng/mL, 27-40; P=0.004) compared with controls (18ng/mL, 11-23), but not in untreated viraemic individuals. No association was observed between CSF sCD30 and plasma HIV-1 RNA, concurrent or nadir CD4+ T cell count, duration of infection or plasma sCD30. CSF sCD30 correlated with CSF NFL (r=0.34, P=0.001).In contrast to plasma, sCD30 levels are elevated in the CSF of individuals with HIV-1 infection who are on suppressive ART. Elevated levels of sCD30 in the CSF may be an indicator of persistent CNS HIV-1 infection, although the mechanism underlying this elevation warrants further investigation.
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18.
  • Rosilio, M, et al. (författare)
  • Long-term improvement of quality of life during growth hormone (GH) replacement therapy in adults with GH deficiency, as measured by questions on life satisfaction-hypopituitarism (QLS-H)
  • 2004
  • Ingår i: Journal of Clinical Endocrinology and Metabolism. - : The Endocrine Society. - 1945-7197 .- 0021-972X. ; 89:4, s. 1684-1693
  • Tidskriftsartikel (refereegranskat)abstract
    • Questions on Life Satisfaction-Hypopituitarism (QLS-H) is a new quality-of-life (QoL) questionnaire developed for adults with hypopituitarism. To determine the effects of long-term GH treatment on QoL, we evaluated QLS-H Z-scores in 576 adult patients with GH deficiency (GHD) enrolled in HypoCCS, an international observational study, using data from five countries in which comparative QLS-H data from the general population were available. Baseline QLS-H Z-scores were significantly lower in GH-deficient patients than in the general population of the same age, gender, and nationality. Z-scores were also significantly lower in female patients vs. males (P=0.006) and in adult-onset vs. childhood-onset GHD (P=0.002). Multivariate analysis associated female gender, multiple pituitary hormone deficiencies, low serum IGF-I values (<75 μg/liter), and concomitant antidepressant medication with low baseline Z-scores. QLS-H Z-scores increased from -1.02&PLUSMN;1.43 (SD) at baseline to -0.25&PLUSMN;1.34 (SD) after 1 yr of GH treatment (P<0.001) and were no longer significantly different from the general population after 4 yr of treatment. There was no correlation between change in Z-score and GH dose or changes in IGF-I and IGF binding protein-3 during treatment. This study demonstrates that 1) improvements in QoL, as measured by the QLS-H, are maintained during long-term GH replacement therapy of adults with GHD, and 2) the QLS-H is a useful tool for evaluating QoL in hypopituitary patients treated in clinical practice. The authors suggest that evaluation of QoL should be a part of the routine clinical management of adult GH-deficient patients, complementing the measurement of surrogate biological markers or other clinical end points.
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19.
  • Spagnoletti, P., et al. (författare)
  • Coulomb excitation of Rn 222
  • 2022
  • Ingår i: Physical Review C. - 2469-9985. ; 105:2
  • Tidskriftsartikel (refereegranskat)abstract
    • The nature of quadrupole and octupole collectivity in Rn222 was investigated by determining the electric-quadrupole (E2) and octupole (E3) matrix elements using subbarrier, multistep Coulomb excitation. The radioactive Rn222 beam, accelerated to 4.23 MeV/u, was provided by the HIE-ISOLDE facility at CERN. Data were collected in the Miniball γ-ray spectrometer following the bombardment of two targets, Sn120 and Ni60. Transition E2 matrix elements within the ground-state and octupole bands were measured up to 10ℏ and the results were consistent with a constant intrinsic electric-quadrupole moment, 518(11)efm2. The values of the intrinsic electric-octupole moment for the 0+→3- and 2+→5- transitions were found to be respectively 2360-210+300efm3 and 2300-500+300efm3 while a smaller value, 1200-900+500efm3, was found for the 2+→1- transition. In addition, four excited non-yrast states were identified in this work via γ-γ coincidences.
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