Myelinosome Organelles in the Retina of R6/1 Huntington Disease (HD) Mice: Ubiquitous Distribution and Possible Role in Disease Spreading

Visual deficit is one of the complications of Huntington disease (HD), a fatal neurological disorder caused by CAG trinucleotide expansions in the Huntingtin gene, leading to the production of mutant Huntingtin (mHTT) protein. Transgenic HD R6/1 mice expressing human HTT exon1 with 115 CAG repeats recapitulate major features of the human pathology and exhibit a degeneration of the retina. Our aim was to gain insight into the ultrastructure of the pathological HD R6/1 retina by electron microscopy (EM). We show that the HD R6/1 retina is enriched with unusual organelles myelinosomes, produced by retinal neurons and glia. Myelinosomes are present in all nuclear and plexiform layers, in the synaptic terminals of photoreceptors, in the processes of retinal neurons and glial cells, and in the subretinal space. In vitro study shows that myelinosomes secreted by human retinal glial Müller MIO-M1 cells transfected with EGFP-mHTT-exon1 carry EGFP-mHTT-exon1 protein, as revealed by immuno-EM and Western-blotting. Myelinosomes loaded with mHTT-exon1 are incorporated by naive neuronal/neuroblastoma SH-SY5Y cells. This results in the emergence of mHTT-exon1 in recipient cells. This process is blocked by membrane fusion inhibitor MDL 28170. Conclusion: Incorporation of myelinosomes carrying mHTT-exon1 in recipient cells may contribute to HD spreading in the retina. Exploring ocular fluids for myelinosome presence could bring an additional biomarker for HD diagnostics.     

Evaluation of dynamic modulus measurement for C/C-SiC composites at different temperatures

The determination of elastic properties at application temperature is fundamental for the design of fibre reinforced ceramic composite components. An attractive method to characterize the flexural modulus at room and high temperature under specific atmosphere is the nondestructive Resonant Frequency Damping Analysis (RFDA). The objective of this paper was to evaluate and validate the modulus measurement via RFDA for orthotropic C/C-SiC composites at the application temperature. At room temperature flexural moduli of C/C-SiC with 0/90° reinforcement were measured under quasi-static 4-point bending loads and compared with dynamic moduli measured via RFDA longitudinally to fibre direction. The dynamic modulus of C/C-SiC was then measured via RFDA up to 1250°C under flowing inert gas and showed an increase with temperature which fitted with literature values. The measured fundamental frequencies were finally compared to those resulting from numerical modal analyses. Dynamic and quasi-static flexural moduli are comparable and the numerical analyses proved that bending modes are correctly modeled by means of dynamic modulus measured via RFDA. The nondestructive RFDA as well as the numerical modeling approach are suitable for evaluation of C/C-SiC and may be transferred to other fibre reinforced ceramic composite materials.     

Multidimensional characterization,Landau levels and Density of States in epitaxial graphene grown on SiC substrates

Using high-temperature annealing conditions with a graphite cap covering the C-face of, both, on axis and 8° off-axis 4H-SiC samples, large and homogeneous single epitaxial graphene layers have been grown. Raman spectroscopy shows evidence of the almost free-standing character of these monolayer graphene sheets, which was confirmed by magneto-transport measurements. On the best samples, we find a moderate p-type doping, a high-carrier mobility and resolve the half-integer quantum Hall effect typical of high-quality graphene samples. A rough estimation of the density of states is given from temperature measurements.     

Refractory concretes uniaxial compression behaviour under high temperature testing conditions

Two refractory concretes with the same matrix composition, one based on andalusite aggregate and the other on bauxite aggregate, have been studied at temperatures ranging from room temperature to 1200 °C maximal temperature. Samples dried at 110 °C during 24 h to create dried standard specimens and others, fired at different temperatures, were subjected to several uniaxial compression test conditions conducted at various temperatures. The evolution of the materials’ global behaviour from quasi-brittle to viscous was evidenced and correlated to their microstructure evolution. Loading/unloading, creep, and strain rate jump tests helped define the damageable and viscoplastic nature of the behaviour. The influence of the firing temperature and duration on the materials’ behaviour is also reported and discussed. In conclusion, potential constitutive equations that are able to fit the material recorded behaviour are suggested.     

Crystal chemistry of the monazite structure

The AXO₄ monazite-type compounds form an extended family that is described in this review in terms of field of stability versus composition. All the substitution possibilities on the cationic and anionic sites leading to the monazite structure are reported. The phosphate, vanadate, chromate, arseniate, sulphate and silicate families are described and the unit-cell parameters of pure compounds and solid solutions are gathered. The stability limits of the monazite-type structure are discussed versus several models generally correlated with geometric criteria. The effects of physico-chemical parameters such as pressure, temperature and irradiation on the monazite-type structure stability are also discussed. The structural relationships between the monazite structure and the related structures (zircon, anhydrite, barite, AgMnO₄, scheelite and monoclinic BiPO₄, CaSeO₃, rhabdophane and SrNp(PO₄)₂) are described.     

Silver coated aluminium microrods as highly colloidal stable SERS platforms

We report on the fabrication of a novel material with the ability to remain in solution even under the very demanding conditions required for structural and dynamic characterization of biomacromolecule assays. This stability is provided by the increase in surface area of a low density material (aluminium) natively coated with a very hydrophilic surface composed of aluminium oxide (Al(2)O(3)) and metallic silver nanoparticles. Additionally, due to the dense collection of active hot spots on their surface, this material offers higher levels of SERS intensity as compared with the same free and aggregated silver nanoparticles.     

Optimization of post-combustion CO2 process using DEA-MDEA mixtures

This paper presents optimal operating conditions for the post-combustion CO₂ capture process utilizing aqueous amine solutions obtained using a process simulator (HYSYS). Three alkanolamine solutions (Methyldiethanolamine MDEA, DiEthanolAmine DEA and MDEA-DEA mixture) are considered to study the performance of the capture process.The design problem addressed in this paper requires specifying the optimal operating conditions (inlet and outlet temperature of the lean solution stream on the absorber, CO₂ loading, amine composition and flow rates, among others) to achieve the given CO₂ emission targets at a minimum total annual cost. A detailed objective function including total operating costs and investment is considered.The influence of the variation of CO₂ reduction targets and the mixing proportion of amines on the total annual cost is analyzed in detail. Numerical results are presented and discussed using different case studies.The results demonstrate that process simulators can be used as a powerful tool not only to simulate but also to optimize the most important design parameters of the post-combustion CO₂ capture process.     

Effects of the nature of the doping salt and of the thermal pre-treatment and sintering temperature on Spark Plasma Sintering of transparent alumina

A slurry of α-Al₂O₃ was doped with Mg, Zr and La nitrates or chlorides, in various amounts in the range 150-500 wt ppm and then freeze-dried to produce nanosized doped powder (∼150 nm). The powder was sintered by SPS to yield transparent polycrystalline alpha alumina. The influence of the nature of the doping element and the starting salt, the thermal treatment before sintering and the sintering temperature on the transparency of the ceramics were investigated. The transparency of the ceramics of nanosized Al₂O₃ was shown to depend mainly on the way the powder was prepared, the nature of the doping salt also had an effect. Finally, a high real inline transmittance, reaching 48.1% was achieved after optimization.     

Inulin mixed esters crosslinked with 2-ethyl-hexyl-acrylate and their promotion as bio-based materials

New inulin-based materials were obtained by graft-copolymerization of inulin (poly-β(1 → 2)-fructoside) with 2-ethyl-hexyl acrylate. Inulin mixed esters were first synthesized by acylation using methacryloyl and palmitoyl chlorides. Further, these esters were copolymerized with 2-ethyl-hexyl acrylate in order to obtain bio-based crosslinked materials that could be used as commodity plastics and that would have biodegradable properties. The obtained products were characterized using FT-IR and ¹H NMR spectroscopies, thermogravimetry; differential scanning calorimetry, and activation energy for the degradation processes (using Kissinger method) was calculated. These biomaterials were also subjected to density measurement, tensile and torsion tests to evaluate their mechanical properties.