Superior Cerebellar Atrophy: An Imaging Clue to Diagnose ITPR1-Related Disorders

The inositol 1,4,5-triphosphate receptor type 1 (ITPR1) gene encodes an InsP₃-gated calcium channel that modulates intracellular Ca²⁺ release and is particularly expressed in cerebellar Purkinje cells. Pathogenic variants in the ITPR1 gene are associated with different types of autosomal dominant spinocerebellar ataxia: SCA15 (adult onset), SCA29 (early-onset), and Gillespie syndrome. Cerebellar atrophy/hypoplasia is invariably detected, but a recognizable neuroradiological pattern has not been identified yet. With the aim of describing ITPR1-related neuroimaging findings, the brain MRI of 14 patients with ITPR1 variants (11 SCA29, 1 SCA15, and 2 Gillespie) were reviewed by expert neuroradiologists. To further evaluate the role of superior vermian and hemispheric cerebellar atrophy as a clue for the diagnosis of ITPR1-related conditions, the ITPR1 gene was sequenced in 5 patients with similar MRI pattern, detecting pathogenic variants in 4 of them. Considering the whole cohort, a distinctive neuroradiological pattern consisting in superior vermian and hemispheric cerebellar atrophy was identified in 83% patients with causative ITPR1 variants, suggesting this MRI finding could represent a hallmark for ITPR1-related disorders.     

Exact GPS Simulation and Optimal Fair Scheduling With Logarithmic Complexity

Generalized processor sharing (GPS) is a fluid scheduling policy providing perfect fairness over both constant-rate and variable-rate links. The minimum deviation (lead/lag) with respect to the GPS service achievable by a packet scheduler is one maximum packet size. To the best of our knowledge, the only packet scheduler guaranteeing the minimum deviation is worst-case fair weighted fair queueing , which requires on-line GPS simulation. Existing algorithms to perform GPS simulation have worst-case computational complexity per packet transmission (being the number of competing flows). Hence, has been charged for complexity too. However it has been proven that the lower bound complexity to guarantee deviation is, yet a scheduler achieving such a result has remained elusive so far. In this paper, we present L-GPS, an algorithm that performs exact GPS simulation with worst-case complexity and small constants. As such it improves the complexity of all the packet schedulers based on GPS simulation. We also present , an implementation of based on L-GPS. has complexity with small constants, and, since it achieves the minimum possible deviation, it does match the aforementioned complexity lower bound. Furthermore, both L-GPS and comply with constant-rate as well as variable-rate links. We assess the effectiveness of both algorithms by simulating real-world scenarios.     

Photorefractive polarization couplers in elliptical core fibres

Photorefractive polarization couplers written internally in germanium-doped elliptical core fibers at 488, 514, and 532 nm are reported. Complete power transfer between the orthogonal polarization modes of the fiber was achieved for couplers written at 514 and 488 nm, respectively. It is shown that the couplers are nonuniform in length because of the high photoinduced attenuation and also due to two-photon absorption. Polarization coupling of higher order modes is also demonstrated at shorter wavelengths where their polarization beat lengths match the polarization beat length of the fundamental mode at which the coupler was written.<>     

High growth rate process in a SiC horizontal CVD reactor using HCl

The results of a new epitaxial process using an industrial 6 × 2″ wafer reactor with the introduction of HCl during the growth have been reported. A complete reduction of silicon nucleation in the gas phase has been observed even for high silicon dilution parameters (Si/H₂ > 0.05%) and an increase of the growth rate until about 20 μm/h has been measured. Photoluminescence at room temperature and at 50 K was used for defects quantification and distribution. On these wafers grown using HCl high voltage Schottky diodes have been realized. The diodes were analyzed by current-voltage (I-V) characteristics.     

Structural and Catalytic Studies of a Trimethyltin Vanadate Coordination Polymer

The organotin vanadate [Me₃SnVO₃] (1) has been prepared and characterised in the solid state by powder X-ray diffraction (XRD), thermogravimetric analysis, multinuclear magic-angle spinning (MAS) NMR, IR and Raman spectroscopy. The phase purity and structure of microcrystalline 1 were confirmed by carrying out a full Rietveld structural refinement at ambient temperature and from conventional powder XRD. ⁵¹V and ¹¹⁹Sn MAS NMR data for compound 1 were in agreement with the predicted structure, showing two equally-abundant, nonequivalent Me₃Sn groups and two equally-abundant, nonequivalent vanadium atoms. The compound was applied as a catalyst for the liquid-phase epoxidation of olefins at 55 °C using tert-butyl hydroperoxide (tBuOOH) as the oxidant. The reaction rate for the different substrates followed the order cis-cyclooctene > (R)-(+)-limonene ≅ trans-2-octene > cyclododecene > styrene > 1-octene; the corresponding epoxides were the only observed products. Leaching tests indicated that the catalytic epoxidation of cyclooctene was mainly heterogeneous in nature. This paper is dedicated to Professor Ian Manners and his scientific accomplishments.     

New synthesis technique of supported ZSM-5 using organo-alumino-silicic gels

This contribution deals with the synthesis of a silicon-rich ZSM-5 obtained from an amorphous organo-alumino-silicic gel. The gel was formed by reacting a mechanical mixture of RHA (rice hull ash), silica’s source, and of natural clinoptilolite, source of alumina, with glycerol during 2 h at 200 °C. After a maturation period of one day at 60 °C, the reaction product was hydrolyzed by a tetra-propyl-ammonium (TPA) solution and then aged for two days at 135 °C in a stainless steel autoclave. After washing and drying, the X-ray pattern showed a well crystallized ZSM-5. The textural analysis of the slowly calcined solid at 500 °C for 4 h, and the SEM micrographies evidence that the zeolite was supported on mesoporous silica. The proportion of both components depends on the tunable hydrolysis conditions.     

Incorporation of polyfluorenes into poly(lactic acid) films for sensor and optoelectronics applications

Films of neat and plasticized biodegradable poly(lactic acid) (PLA) matrices containing anionic conjugated polyelectrolytes, poly[9,9‐bis(4‐phenoxybutylsulfonate)]fluorene‐2,7‐diyl‐alt‐arylenes, with 1,4‐phenylene and 4,4″‐p‐terphenylene, respectively, as arylene groups or a neutral poly(9,9‐dialkylfluorene) for comparison were prepared by solution casting. These films were characterized using differential scanning calorimetry, thermogravimetry, scanning electron microscopy and fluorescence spectroscopy. In addition, the effects of plasticizer on the thermal properties and the oxygen permeability of the PLA films were measured through the oxygen transmission rate. Results show that it is possible to obtain thin, optically transparent and luminescent films with potential in oxygen sensing, exhibiting good thermal and photochemical stability. At high polyelectrolyte content, evidence is found for phase separation and aggregate formation and it is no longer possible to obtain completely homogeneous films. The possibility of incorporating the cationic metal complex tris(2,2′‐bipyridyl)ruthenium(II) into plasticized PLA films containing conjugated polyelectrolytes for dual‐wavelength ratiometric luminescence sensing is also discussed. Copyright © 2012 Society of Chemical Industry     

Hydrogen Peroxide-Preconditioned Human Adipose-Derived Stem Cells Enhance the Recovery of Oligodendrocyte-Like Cells after Oxidative Stress-Induced Damage

Oxidative stress associated with neuroinflammation is a key process involved in the pathophysiology of neurodegenerative diseases, and therefore, has been proposed as a crucial target for new therapies. Recently, the therapeutic potential of human adipose-derived stem cells (hASCs) has been investigated as a novel strategy for neuroprotection. These cells can be preconditioned by exposing them to mild stress in order to improve their response to oxidative stress. In this study, we evaluate the therapeutic potential of hASCs preconditioned with low doses of H₂O₂ (called HC016 cells) to overcome the deleterious effect of oxidative stress in an in vitro model of oligodendrocyte-like cells (HOGd), through two strategies: i, the culture of oxidized HOGd with HC016 cell-conditioned medium (CM), and ii, the indirect co-culture of oxidized HOGd with HC016 cells, which had or had not been exposed to oxidative stress. The results demonstrated that both strategies had reparative effects, oxidized HC016 cell co-culture being the one associated with the greatest recovery of the damaged HOGd, increasing their viability, reducing their intracellular reactive oxygen species levels and promoting their antioxidant capacity. Taken together, these findings support the view that HC016 cells, given their reparative capacity, might be considered an important breakthrough in cell-based therapies.     

Diffusion of electrolytes in hydrolyzable glassy polymers: Acetic acid in poly(vinyl acetate), poly(vinyl alcohol), and polyesters

Engineering materials containing poly(vinyl acetate) (PVAc) as the key component undergo hydrolytic degradation, which must be minimized or, at least, controlled. To characterize PVAc hydrolysis quantitatively, the diffusion of acetic acid (HAc) in PVAc, poly(vinyl alcohol) (PVA), unsaturated polyester (UPE), and a UPE/PVAc blend was studied in detail. The permeability cell earlier developed by the authors was modified here to reduce experimental error. As the diffusion and solubility coefficients of water and HAc in the above materials were measured at different temperatures, a mathematical model was developed, which takes proper account of the combined water and HAc diffusion in PVAc undergoing partial hydrolysis. The model was further validated by the experimental data obtained at 70°C for UPE/PVAc film, simulating a matrix of sheet‐molding compounds composite materials. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 83: 1157–1166, 2002