37 research outputs found

    Structure of Kα1,2 - And Kβ1,3 -emission x-ray spectra for Se, Y, and Zr

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    UID/FIS/04559/2020 UID/MULTI/04046/2020 Project No. PTDC/FIS-AQM/31969/20 Grant No. 2017/25/B/ST2/00901The Kα and Kβ x-ray spectra of Se, Y, and Zr were studied experimentally and theoretically in order to obtain information on the Kα1 line asymmetry and the spin doublet in Kβ1,3 diagram lines. Using a high-resolution antiparallel double-crystal x-ray spectrometer, we obtained the line shapes, that is, asymmetry index and natural linewidths. We found that the corrected full width at half maximum of the Kα1 and Kα2 lines as a function of Z is in good agreement with the data in the literature. Furthermore, satellite lines arising from shake-off appear in the low-energy side of the Kα1 and Kα2 lines in Se but, in Y and Zr, it was very difficult to identify the contribution of the shake process to the overall lines. The Kβ1,3 natural linewidth of these elements was also corrected using the appropriate instrumental function for this type of x-ray spectrometer, and the spin doublet energies were obtained from the peak positions. The corrected full width at half maximum (FWHM) of the Kβ1 x-ray lines increases linearly with Z, but this tendency was found to be, in general, not linear for Kβ3 x-ray lines. This behavior may be due to the existence of satellite lines originated from shake processes. Simulated line profiles, obtained using the multiconfiguration Dirac-Fock formalism, accounting for radiative and radiationless transitions and shake-off processes, show a very good agreement with the high-resolution experimental spectra.publishersversionpublishe

    High LRRK2 Levels Fail to Induce or Exacerbate Neuronal Alpha-Synucleinopathy in Mouse Brain

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    The G2019S mutation in the multidomain protein leucine-rich repeat kinase 2 (LRRK2) is one of the most frequently identified genetic causes of Parkinson’s disease (PD). Clinically, LRRK2(G2019S) carriers with PD and idiopathic PD patients have a very similar disease with brainstem and cortical Lewy pathology (α-synucleinopathy) as histopathological hallmarks. Some patients have Tau pathology. Enhanced kinase function of the LRRK2(G2019S) mutant protein is a prime suspect mechanism for carriers to develop PD but observations in LRRK2 knock-out, G2019S knock-in and kinase-dead mutant mice suggest that LRRK2 steady-state abundance of the protein also plays a determining role. One critical question concerning the molecular pathogenesis in LRRK2(G2019S) PD patients is whether α-synuclein (aSN) has a contributory role. To this end we generated mice with high expression of either wildtype or G2019S mutant LRRK2 in brainstem and cortical neurons. High levels of these LRRK2 variants left endogenous aSN and Tau levels unaltered and did not exacerbate or otherwise modify α-synucleinopathy in mice that co-expressed high levels of LRRK2 and aSN in brain neurons. On the contrary, in some lines high LRRK2 levels improved motor skills in the presence and absence of aSN-transgene-induced disease. Therefore, in many neurons high LRRK2 levels are well tolerated and not sufficient to drive or exacerbate neuronal α-synucleinopathy

    Multi-messenger observations of a binary neutron star merger

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    On 2017 August 17 a binary neutron star coalescence candidate (later designated GW170817) with merger time 12:41:04 UTC was observed through gravitational waves by the Advanced LIGO and Advanced Virgo detectors. The Fermi Gamma-ray Burst Monitor independently detected a gamma-ray burst (GRB 170817A) with a time delay of ~1.7 s with respect to the merger time. From the gravitational-wave signal, the source was initially localized to a sky region of 31 deg2 at a luminosity distance of 40+8-8 Mpc and with component masses consistent with neutron stars. The component masses were later measured to be in the range 0.86 to 2.26 Mo. An extensive observing campaign was launched across the electromagnetic spectrum leading to the discovery of a bright optical transient (SSS17a, now with the IAU identification of AT 2017gfo) in NGC 4993 (at ~40 Mpc) less than 11 hours after the merger by the One- Meter, Two Hemisphere (1M2H) team using the 1 m Swope Telescope. The optical transient was independently detected by multiple teams within an hour. Subsequent observations targeted the object and its environment. Early ultraviolet observations revealed a blue transient that faded within 48 hours. Optical and infrared observations showed a redward evolution over ~10 days. Following early non-detections, X-ray and radio emission were discovered at the transient’s position ~9 and ~16 days, respectively, after the merger. Both the X-ray and radio emission likely arise from a physical process that is distinct from the one that generates the UV/optical/near-infrared emission. No ultra-high-energy gamma-rays and no neutrino candidates consistent with the source were found in follow-up searches. These observations support the hypothesis that GW170817 was produced by the merger of two neutron stars in NGC4993 followed by a short gamma-ray burst (GRB 170817A) and a kilonova/macronova powered by the radioactive decay of r-process nuclei synthesized in the ejecta

    Parkinson's Disease: Basic Pathomechanisms and a Clinical Overview

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    PD is a common and a debilitating degenerative movement disorder. The number of patients is increasing worldwide and as yet there is no cure for the disease. The majority of existing treatments target motor symptom control. Over the last two decades the impact of the genetic contribution to PD has been appreciated. Significant discoveries have been made, which have advanced our understanding of the pathophysiological and molecular basis of PD. In this chapter we outline current knowledge of the clinical aspects of PD and the basic mechanistic understanding

    Structural basis of chaperone–subunit complex recognition by the type 1 pilus assembly platform FimD

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    Adhesive type 1 pili from uropathogenic Escherichia coli are filamentous protein complexes that are attached to the assembly platform FimD in the outer membrane. During pilus assembly, FimD binds complexes between the chaperone FimC and type 1 pilus subunits in the periplasm and mediates subunit translocation to the cell surface. Here we report nuclear magnetic resonance and X-ray protein structures of the N-terminal substrate recognition domain of FimD (FimD(N)) before and after binding of a chaperone–subunit complex. FimD(N) consists of a flexible N-terminal segment of 24 residues, a structured core with a novel fold, and a C-terminal hinge segment. In the ternary complex, residues 1–24 of FimD(N) specifically interact with both FimC and the subunit, acting as a sensor for loaded FimC molecules. Together with in vivo complementation studies, we show how this mechanism enables recognition and discrimination of different chaperone–subunit complexes by bacterial pilus assembly platforms

    The IAEA CRP on Studies of Advanced Reactor Technology Options for Effective Incineration of Radioactive Waste

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    In 2003, the IAEA has initiated the Coordinated Research Project (CRP) on ¿Studies of Advanced Reactor Technology Options for Effective Incineration of Radioactive Waste¿. The overall objective of the CRP, performed within the framework of IAEA¿s Nuclear Energy Department¿s Technical Working Group on Fast Reactors, is to increase the capability of Member States in developing and applying advanced technologies in the area of long-lived radioactive waste utilization and transmutation. Twenty institutions from 15 Member States and one international organization participated in this CRP. The CRP concentrated on the assessment of the dynamic behavior of various transmutation systems. The reactor systems investigated comprise critical reactors, sub-critical accelerator driven systems with heavy liquid metal and gas cooling, critical molten salt systems, and hybrid fusion/fission systems. Both fertile and fertile-free fuel options have been investigated. Apart from the benchmarking of steady state core configurations (including the investigation of transmutation potential, burn-up behavior and decay heat of minor actinide (MA) bearing fuels), the CRP participants determined the safety coefficients for the individual systems and, in a second stage, performed transient analyses which reflected the generic safety related behavior of the various reactors types.JRC.F.4-Safety of future nuclear reactor
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