Functional and Molecular Properties of DYT-SGCE Myoclonus-Dystonia Patient-Derived Striatal Medium Spiny Neurons

Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by mutations in the SGCE gene encoding ε-sarcoglycan leading to a dysfunction of this transmembrane protein, alterations in the cerebello-thalamic pathway and impaired striatal plasticity. To elucidate underlying pathogenic mechanisms, we investigated induced pluripotent stem cell (iPSC)-derived striatal medium spiny neurons (MSNs) from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene (c.298T>G and c.304C>T with protein changes W100G and R102X) in comparison to two matched healthy control lines. Calcium imaging showed significantly elevated basal intracellular Ca²⁺ content and lower frequency of spontaneous Ca²⁺ signals in SGCE MSNs. Blocking of voltage-gated Ca²⁺ channels by verapamil was less efficient in suppressing KCl-induced Ca²⁺ peaks of SGCE MSNs. Ca²⁺ amplitudes upon glycine and acetylcholine applications were increased in SGCE MSNs, but not after GABA or glutamate applications. Expression of voltage-gated Ca²⁺ channels and most ionotropic receptor subunits was not altered. SGCE MSNs showed significantly reduced GABAergic synaptic density. Whole-cell patch-clamp recordings displayed elevated amplitudes of miniature postsynaptic currents and action potentials in SGCE MSNs. Our data contribute to a better understanding of the pathophysiology and the development of novel therapeutic strategies for myoclonus-dystonia.     

In situ esterification of rice bran oil with methanol and ethanol

In situ esterifications of high-acidity rice bran oil with methanol and ethanol and with sulfuric acid as catalyst were investigated. In the esterification with methanol, all free fatty acids (FFA) dissolved in methanol were interesterified within 15 min, and it was possible to obtain nearly pure methyl esters. The amount of methyl esters obtained from a given rice bran was dependent on the FFA content of the rice bran oil. In the esterification with ethanol, it was not possible to obtain pure esters as in methanol esterification, because the solubilities of oil components in ethanol were much higher than those in methanol.     

Disinfection Capacity of High-Power Ultrasound Against E. coli O157:H7 in Process Water of the Fresh-Cut Industry

The fresh-cut industry must treat process water to guarantee its microbial quality before reuse or recirculation back into the processing line. In the present study, the suitability of high-power ultrasound (HPU) for disinfecting and recycling process water was evaluated. An ultrasonic horn (20 kHz) was used to inactivate Escherichia coli O157:H7 inoculated in five types of process water which showed different physical and chemical characteristics. Differences in the inactivation level of E. coli O157:H7 at different HPU densities (0.14, 0.28, 0.56, and 1.12 kW/L) with controlled (20–25 °C) and uncontrolled (15–72 °C, 3.6 °C/min) temperature increase were studied. Results showed that the higher the power density and temperature, the higher the efficiency, reaching up to 6 log reductions of E. coli O157:H7. Alkalinity (between 0 and 253 mg HCO₃ ^?/L) and organic matter concentration (between 9 and 3,525 mg O₂/L) in water did not reduce ultrasonic efficacy against E. coli O157:H7. Agglomerates >90 μm, which represented 34 % of those present in the process water, were reduced to only 11 % by HPU. Results indicate that HPU can be successfully applied to treat process water of the fresh produce industry because the antimicrobial efficacy was not affected by the continuous variation of the process water quality. HPU can be a suitable technology for the fresh produce industry to be able to reduce consumption of water and decrease wastewater and the generation of disinfection by-products.     

CFD and experimental investigation of the gas–liquid flow in the distributor of a compact heat exchanger

High performance of compact heat exchangers is conditioned by correct fluid distribution. This is especially true for gas–liquid heat exchangers where a uniform distribution is particularly delicate to obtain and where maldistribution entails significant performance deterioration. Several phenomena can lead to phase distribution problems: the fins may be subject to manufacturing defects or fouling, leading to shortcuts or dead zones. But the first source of maldistribution may be a poor distribution at the outlet of the entrance distributor. This distributor aims at mixing the phases and distributing them across the channels.     

Characterization of glow‐discharge–treated cellulose acetate membrane surfaces for single‐layer enzyme electrode studies

Cellulose acetate membranes (CA) were modified by means of plasma polymerization of ethylene diamine (EDA) and n‐butylamine (n‐BA). The motivation for this work was the application of a modified membrane for the single‐layer enzyme electrode. A tubular reactor with the external radiofrequency (13.56 MHz) excitation was used. Surface modification was performed at 5, 10, and 15 W power (at 27 Pa working pressure) for 5, 10, 15 min. Modified surfaces were characterized in detail by FTIR–ATR, XPS (ESCA), contact angle, and enzyme immobilization activity. The best treatment results were obtained for EDA with 5 W and 30 min and 15 W and 10 min. These results are discussed using surface analysis data. © 2001 John Wiley & Sons, Inc. J Appl Polym Sci 81: 1341–1352, 2001     

Prolonged rewarding stimulation of the rat medial forebrain bundle: Neurochemical and behavioral consequences.

Extracellular dopamine levels were measured in the rat nucleus accumbens by means of in vivo microdialysis. Delivery of rewarding medial forebrain bundle stimulation at a low rate (5 trains/min) produced a sustained elevation of dopamine levels, regardless of whether train onset was predictable. When the rate of train delivery was increased to 40 trains/min, dopamine levels rose rapidly during the first 40 min but then declined toward the baseline range. The rewarding impact of the stimulation was reduced following prior delivery of stimulation at the high, but not the low, rate. These results support the idea that dopamine tone plays an enabling role in brain stimulation reward and is elevated similarly by predictable and unpredictable stimulation. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Adaptive channel allocation for wireless PCN

In cellular networks, forced call terminations due to handoff call blocking are generally more objectionable than new call blocking. In order to maintain an acceptable call dropping probability rate, we propose, in this paper, two new guard channel schemes: an adaptive one – New Adaptive Channel Reservation (NACR) – and a dynamic one – Predictive Reservation Policy (PRP). In NACR, for a given period of time, a given number of channels is guarded in each cell for handoff traffic. In PRP, the number of reserved channels depends on the actual number of calls in progress in the neighboring cells. An approximate analytical model of NACR is presented. A Tabu search method has been implemented in order to optimize the Grade of Service. Discrete event simulations of PRP and NACR were run. The effectiveness of the proposed methods is emphasized on a complex configuration.     

The MISTIGRI thermal infrared project: scientific objectives and mission specifications

This article presents the MISTIGRI project of a microsatellite developed by the French space agency Centre National d'Etudes Spatiales (CNES) in cooperation with Spain (Image Processing Laboratory of the University of Valencia and Centro para el Desarrollo Tecnológico Industrial (CDTI)). MISTIGRI is a mission that has the originality of combining a high spatial resolution (～50 m) with a daily revisit in the thermal infrared (TIR). MISTIGRI is an experimental mission devoted to demonstrate the potential of such TIR data for future operational missions. The scientific goals and expected applications of the mission are described: they encompass the monitoring of (i) agricultural areas and related hydrological processes, (ii) urban areas, and (iii) coastal areas and continental waters. Then, the specifications on spatial resolution, revisit frequency, overpass time, and spectral configuration are justified. The strategy of the mission is based on the combination with a network of long-term experimental sites. It will also make possible observing some areas facing rapid climatic change. The choice of the orbit is presented. Finally, we give rapid overviews of both the instrumental concept and the proposed mission architecture.