94 research outputs found
Revealing sub-{\mu}m inhomogeneities and {\mu}m-scale texture in H2O ice at Megabar pressures via sound velocity measurements by time-domain Brillouin scattering
Time-domain Brillouin scattering technique, also known as picosecond
ultrasonic interferometry, which provides opportunity to monitor propagation of
nanometers to sub-micrometers length coherent acoustic pulses in the samples of
sub-micrometers to tens of micrometers dimensions, was applied to
depth-profiling of polycrystalline aggregate of ice compressed in a diamond
anvil cell to Megabar pressures. The technique allowed examination of
characteristic dimensions of elastic inhomogeneities and texturing of
polycrystalline ice in the direction normal to the diamond anvil surfaces with
sub-micrometer spatial resolution via time-resolved measurements of variations
in the propagation velocity of the acoustic pulse traveling in the compressed
sample. The achieved two-dimensional imaging of the polycrystalline ice
aggregate in-depth and in one of the lateral directions indicates the
feasibility of three-dimensional imaging and quantitative characterization of
acoustical, optical and acousto-optical properties of transparent
polycrystalline aggregates in diamond anvil cell with tens of nanometers
in-depth resolution and lateral spatial resolution controlled by pump laser
pulses focusing.Comment: 32 pages, 5 figure
Electronic Band Transitions in Îł-Ge3N4
This work has been carried out within the framework of the EUROfusion Consortium and has received funding from the Euratom research and training programme 2014-2018 and 2019-2020 under grant agreement No 633053. The views and opinions expressed herein do not necessarily reflect those of the European Commission. Support from Estonian Research Council grant PUT PRG 619 is gratefully acknowledged. The multi-anvil experiments at LMV were supported by the French Government Laboratory of Excellence initiative no ANR-10-LABX-0006, the RĂ©gion Auvergne and the European Regional Development Fund (ClerVolc Contribution Number 478).Electronic band structure in germanium nitride having spinel structure, Îł-Ge3N4, was examined using two spectroscopic techniques, cathodoluminescence and synchrotron-based photoluminescence. The sample purity was confirmed by x-ray diffraction and Raman analyses. The spectroscopic measurements provided first experimental evidence of a large free exciton binding energy Deâ0.30 eV and direct interband transitions in this material. The band gap energy Eg = 3.65 ± 0.05 eV measured with a higher precision was in agreement with that previously obtained via XES/XANES method. The screened hybrid functional HeydâScuseriaâErnzerhof (HSE06) calculations of the electronic structure supported the experimental results. Based on the experimental data and theoretical calculations, the limiting efficiency of the excitation conversion to light was estimated and compared with that of w-GaN, which is the basic material of commercial light emitting diodes. The high conversion efficiency, very high hardness and rigidity combined with a thermal stability in air up to ~ 700 °C reveal the potential of Îł-Ge3N4 for robust and efficient photonic emitters. © 2021, The Korean Institute of Metals and Materials. Published under the CC BY license.Euratom research and training programme 2014-2018 633053; Eesti Teadusagentuur ANR-10-LABX-0006, PUT PRG 619; ERDF; Institute of Solid State Physics, University of Latvia as the Center of Excellence has received funding from the European Unionâs Horizon 2020 Framework Programme H2020-WIDESPREAD-01-2016-2017-TeamingPhase2 under grant agreement No. 739508, project CAMART2
Single-crystal elastic moduli, anisotropy and the B1-B2 phase transition of NaCl at high pressures: Experiment vs. ab-initio calculations
Single-crystal elastic moduli, Cij, and the B1-B2 phase transition of NaCl
were investigated experimentally, using time-domain Brillouin scattering
(TDBS), and theoretically, via density-functional-theory (DFT), to 41 GPa.
Thus, we largely extended pressure range where Cij and elastic anisotropy of
the solid are measured, including the first experimental data for the
high-pressure B2 phase, NaCl-B2. NaCl-B1 exhibits a strong and growing with
pressure anisotropy, in contrast to NaCl-B2. Theoretical values obtained using
different advanced DFT functionals were compared with our measurements but no
one could satisfactorily reproduce our experimental data for NaCl-B1 and
NaCl-B2 simultaneously. For all available DFT results on the principal shear
moduli and anisotropy, the deviation became pronounced when the degree of
compression increased significantly. Similar deviations could be also
recognized for other cubic solids having the same B1-type structure and similar
bonding, such as CaO, MgO, or (Mg1-x,Fex)O. Furthermore, the available
experimental data suggest that the B1-B2 phase transition of NaCl and the above
mentioned compounds are governed by the Born stability criterion C44(P) - P >
0.Comment: 15 pages and 5 figures for the Manuscript, 10 pages and 7 figures for
the Supplemen
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Prospective CERAD Neuropsychological Assessment in Patients With Multiple System Atrophy
The objective of the study was to characterize the pattern of cognitive dysfunction in patients with multiple system atrophy (MSA) applying a standardized neuropsychological assessment. A total of 20 patients with the diagnosis of probable or possible MSA were enrolled for neuropsychological assessment applying the CERAD plus battery. All patients were tested at baseline and 14/20 patients received additional follow-up assessments (median follow-up of 24 months). Additionally, relationship between cortical thickness values/subcortical gray matter volumes and CERAD subitems was evaluated at baseline in a subgroup of 13/20 patients. Trail Making Test (TMT) was the most sensitive CERAD item at baseline with abnormal performance (z-score < â1.28) in one or both pathological TMT items (TMT-A, TMT-B) in 60% of patients with MSA. Additionally, there was a significant inverse correlation between the volume of the left and the right accumbens area and the TMT A item after adjusting for age (left side: p = 0.0009; right side p = 0.003). Comparing both subtypes, patients with MSA-C had significant lower values in phonemic verbal fluency (p = 0.04) and a trend for lower values in semantic verbal fluency (p = 0.06) compared to MSA-P. Additionally, patients with MSA-C showed significantly worse performance in the TMT-B task (p = 0.04) and a trend for worse performance in the TMT-A task (p = 0.06). Concerning longitudinal follow-up, a significant worsening in the TMT-B (p = 0.03) can be reported in MSA. In conclusion, frontal-executive dysfunction presents the hallmark of cognitive impairment in MSA
Novel mutation of the PRNP gene of a clinical CJD case
BACKGROUND: Transmissible spongiform encephalopathies (TSEs), a group of neurodegenerative diseases, are thought to be caused by an abnormal isoform of a naturally occurring protein known as cellular prion protein, PrP(C). The abnormal form of prion protein, PrP(Sc )accumulates in the brain of affected individuals. Both isoforms are encoded by the same prion protein gene (PRNP), and the structural changes occur post-translationally. Certain mutations in the PRNP gene result in genetic TSEs or increased susceptibility to TSEs. CASE PRESENTATION: A 70 year old woman was admitted to the hospital with severe confusion and inability to walk. Relatives recognized memory loss, gait and behavioral disturbances over a six month period prior to hospitalization. Neurological examination revealed Creutzfeldt-Jakob disease (CJD) related symptoms such as incontinence, Babinski sign and myoclonus. EEG showed periodic sharp waves typical of sporadic CJD and cerebrospinal fluid analysis (CSF) was positive for the presence of the 14-3-3-protein. As the disease progressed the patient developed akinetic mutism and died in the tenth month after onset of the disease symptoms. Unfortunately, no autopsy material was available. PRNP sequencing showed the occurrence of a point mutation on one allele at codon 193, which is altered from ACC, coding for a threonine, to ATC, encoding an isoleucine (T193I). CONCLUSION: Here we report a novel mutation of the PRNP gene found in an elderly female patient resulting in heterozygosity for isoleucine and threonine at codon 193, in which normally homozygosity for threonine is expected (T193). The patient presented typical clinical symptoms of CJD. EEG findings and the presence of the 14-3-3 protein in the CSF, contributed to CJD diagnosis, allowing the classification of this case as a probable CJD according to the World Health Organization (WHO) accepted criteria
Evidence for a Pathogenic Role of Different Mutations at Codon 188 of PRNP
Clinical and pathological changes in familial Creutzfeldt-Jakob disease (CJD) cases may be similar or indistinguishable from sporadic CJD. Therefore determination of novel mutations in PRNP remains of major importance
Plasma extracellular vesicle tau and TDP-43 as diagnostic biomarkers in FTD and ALS
Minimally invasive biomarkers are urgently needed to detect molecular pathology in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Here, we show that plasma extracellular vesicles (EVs) contain quantifiable amounts of TDP-43 and full-length tau, which allow the quantification of 3-repeat (3R) and 4-repeat (4R) tau isoforms. Plasma EV TDP-43 levels and EV 3R/4R tau ratios were determined in a cohort of 704 patients, including 37 genetically and 31 neuropathologically proven cases. Diagnostic groups comprised patients with TDP-43 proteinopathy ALS, 4R tauopathy progressive supranuclear palsy, behavior variant FTD (bvFTD) as a group with either tau or TDP-43 pathology, and healthy controls. EV tau ratios were low in progressive supranuclear palsy and high in bvFTD with tau pathology. EV TDP-43 levels were high in ALS and in bvFTD with TDP-43 pathology. Both markers discriminated between the diagnostic groups with area under the curve values >0.9, and between TDP-43 and tau pathology in bvFTD. Both markers strongly correlated with neurodegeneration, and clinical and neuropsychological markers of disease severity. Findings were replicated in an independent validation cohort of 292 patients including 34 genetically confirmed cases. Taken together, the combination of EV TDP-43 levels and EV 3R/4R tau ratios may aid the molecular diagnosis of FTD, FTD spectrum disorders and ALS, providing a potential biomarker to monitor disease progression and target engagement in clinical trials.</p
The Priority position paper: protecting Europe's food chain from prions
International audienceBovine spongiform encephalopathy (BSE) created a global European crisis in the 1980s and 90s, with very serious health and economic implications. Classical BSE now appears to be under control, to a great extent as a result of a global research effort that identified the sources of prions in meat and bone meal (MBM) and developed new animal-testing tools that guided policy. Priority ( www.prionpriority.eu ) was a European Union (EU) Framework Program 7 (FP7)-funded project through which 21 European research institutions and small and medium enterprises (SMEs) joined efforts between 2009 and 2014, to conduct coordinated basic and applied research on prions and prion diseases. At the end of the project, the Priority consortium drafted a position paper ( www.prionpriority.eu/Priority position paper) with its main conclusions. In the present opinion paper, we summarize these conclusions. With respect to the issue of re-introducing ruminant protein into the feed-chain, our opinion is that sustaining an absolute ban on feeding ruminant protein to ruminants is essential. In particular, the spread and impact of non-classical forms of scrapie and BSE in ruminants is not fully understood and the risks cannot be estimated. Atypical prion agents will probably continue to represent the dominant form of prion diseases in the near future in Europe. Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models. Similarly, there are now data indicating that the atypical scrapie agent can cross various species barriers. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of its transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. Intensified searching for molecular determinants of the species barrier is recommended, since this barrier is key for important policy areas and risk assessment. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research, also needed to understand mechanisms of prion transmission, replication and how they cause nervous system dysfunction and death. Early detection of prion infection, ideally at a preclinical stage, also remains crucial for development of effective treatment strategies
Comments on âHardness, elasticity, and fracture toughness of polycrystalline spinel germanium nitride and tin nitride,â by M.P. Shemkunas, W.T. Petuskey, A.V.G. Chizmeshya, K. Leinenweber, and G.H. Wolf [J. Mater. Res. 19, 1392 (2004)]: Reestablishing of elastic moduli for Îł-Ge3N4
It will be shown that in the considered paper, a mistake occurred by handling or editing of experimental data for one of two investigated materials, namely, for cubic germanium nitride having spinel structure (Îł-Ge3N4). This mistake led to incorrect values of the shear modulus G0, Youngâs modulus E0, and Poissonâs ratio Îœ0 of this compound. My effort to recover the elastic moduli of Îł-Ge3N4 from the available data gave the following results: G0=124 GPa, E0=326 GPa, and Îœ0=0.32
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