Novel KCND3 Variant Underlying Nonprogressive Congenital Ataxia or SCA19/22 Disrupt KV4.3 Protein Expression and K+ Currents with Variable Effects on Channel Properties

KCND3 encodes the voltage-gated potassium channel K_V4.3 that is highly expressed in the cerebellum, where it regulates dendritic excitability and calcium influx. Loss-of-function K_V4.3 mutations have been associated with dominant spinocerebellar ataxia (SCA19/22). By targeted NGS sequencing, we identified two novel KCND3 missense variants of the K_V4.3 channel: p.S347W identified in a patient with adult-onset pure cerebellar syndrome and p.W359G detected in a child with congenital nonprogressive ataxia. Neuroimaging showed mild cerebellar atrophy in both patients. We performed a two-electrode voltage-clamp recording of K_V4.3 currents in Xenopus oocytes: both the p.G345V (previously reported in a SCA19/22 family) and p.S347W mutants exhibited reduced peak currents by 50%, while no K+ current was detectable for the p.W359G mutant. We assessed the effect of the mutations on channel gating by measuring steady-state voltage-dependent activation and inactivation properties: no significant alterations were detected in p.G345V and p.S347W disease-associated variants, compared to controls. K_V4.3 expression studies in HEK293T cells showed 53% (p.G345V), 45% (p.S347W) and 75% (p.W359G) reductions in mutant protein levels compared with the wildtype. The present study broadens the spectrum of the known phenotypes and identifies additional variants for KCND3-related disorders, outlining the importance of SCA gene screening in early-onset and congenital ataxia.     

Numerical and experimental studies on aluminum sandwich plates of variable thickness

Abstract

The elastic flexural behavior of static deformation and free vibration of sandwich plates of variable thickness is investigated numerically and experimentally. In the analysis, the face plates are treated as Marguerre shells, and the core is assumed to be an antiplane core and to provide resistance to transverse shear and normal stresses only. Displacement continuity conditions are used at the interfaces between face plates and the core to derive the displacement field. Energy formulations are obtained and solved by the isoparametric finite element method. The numerical results are obtained to compare with the results in the existing literature and to show the effects of taper constant and face plate thickness on deflections and natural frequencies. Finally, experimental works based on the method of holographic interferometry are conducted to confirm the theoretical findings. Experimental and numerical data agree quite well in this work.     

Stability and robustness of time‐delay systems

Abstract

Using some properties of matrix measures and matrix's spectral radius, a new stability criterion for a linear time‐delay system is derived. This result is also extended to interval time‐delay systems.     

A branch and bound algorithm for scheduling problem of a robot‐centered manufacturing cell

Abstract

An alternative formulation of the scheduling problem in a robot‐centered manufacturing cell has been described here, which was originally formulated by Lin et al. [7] as a mixed integer programming problem. An efficient procedure based on the branch and bound technique has been proposed. In order to reduce the complexity of the branching procedure, several sequencing rules [4] have been imbedded into the proposed procedure and an integrated algorithm has then been presented. The computational results have indicated the proposed algorithm to be efficient. Finally, conclusions and some suggestions are given.     

Detection for high resolution radars

Abstract

In this paper we discuss detection problems for a high resolution radar. Fluctuation in the target radar cross section usually decreases the probability of detection. However, through integration of cells within range profiles of a high resolution radar, variation of the integrated magnitude with respect to the change of carrier frequency and target aspect becomes much smaller, and this is helpful for improving the probability of detection. Two detection algorithms, the cell integration method and the correlation method, for a high resolution radar are proposed, and their detection performances are compared with that obtained by a conventional low resolution radar. Some theoretical formulations are developed. Simulation results show the effectiveness of the proposed algorithms.     

The production of renewable transportation fuel through fed‐batch and continuous deoxygenation of vegetable oil derived fatty acids over Pd/C catalyst

Renewable fuels such as green diesel from renewable biomass have received attentions because of energy and environmental concerns. Deoxygenation, used to upgrade biomass derived oils has been applied intensively nowadays. To successfully improve this process, fed‐batch and following continuous processes on real biomass derived fatty acids need to be investigated. In this study, liquid‐phase deoxygenation of vegetable oil derived fatty acids was carried out over 5% Pd/C with cofeeding H₂. With sufficient hydrogen, complete hydrogenation of unsaturated fatty acids occurred, leading to successive deoxygenation of the saturated fatty acids with n‐alkane yields of 99% and 88% and average CO₂ selectivity of 94.9% and 94.6% in the fed‐batch and continuous mode, respectively. After hydrogenation is completed, lower than 5% of effective H₂ partial pressure was found to be favorable for the decarboxylation pathway and ideal for deoxygenation of biomass derived fatty acids. Copyright © 2015 John Wiley & Sons, Ltd.     

SNS collaborative learning design: enhancing critical thinking for human–computer interface design

Universal Access in the Information Society - In many cases, classrooms seem to be functioning as well as ever, though the challenges and expectations have changed quite dramatically. This study...     

Experimental study of the burner for FAA fire test: NexGen burner

The NexGen (Sonic) burner is the new burner developed by the Federal Aviation Administration, FAA, to replace old oil burners used for the required fire certification tests on power plant‐related materials, as it provides the capability to control both air and fuel flow rates. During a fire test, the burner is supposed to simulate a certain fire condition, so the flame properties should be robust and repeatable. The NexGen burner can achieve this due to the precise fuel and air controls. However, the current calibration criterion (ISO2685:1998 and AC20‐135) may not be good enough to ensure consistent flame properties. In the presented work, the sensitivity of the burner performance to air and fuel flow rate, as measured by the temperature and heat flux for calibration purposes, was studied. Additionally, the influence of the turbulator and the thermocouple size used for flame calibration was also studied. The impact of varying fuel/air ratio and thermocouple sizes was studied by conducting fire tests on aluminum samples, to show the inadequacies in the current calibration standards.     

Ultrafine Nanoparticle‐Supported Ru Nanoclusters with Ultrahigh Catalytic Activity

The design of an ideal heterogeneous catalyst for hydrogenation reaction is to impart the catalyst with synergetic surface sites active cooperatively toward different reaction species. Herein a new strategy is presented for the creation of such a catalyst with dual active sites by decorating metal and metal oxide nanoparticles with ultrafine nanoclusters at atomic level. This strategy is exemplified by the design and synthesis of Ru nanoclusters supported on Ni/NiO nanoparticles. This Ru‐nanocluster/Ni/NiO‐nanoparticle catalyst is shown to exhibit ultrahigh catalytic activity for benzene hydrogenation reaction, which is 55 times higher than Ru–Ni alloy or Ru on Ni catalysts. The nanoclusters‐on‐nanoparticles are characterized by high‐resolution transmission electron microscope, Cs‐corrected high angle annular dark field‐scanning transmission electron microscopy, elemental mapping, high‐sensitivity low‐energy ion scattering, and X‐ray absorption spectra. The atomic‐scale nanocluster–nanoparticle structural characteristics constitute the basis for creating the catalytic synergy of the surface sites, where Ru provides hydrogen adsorption and dissociation site, Ni acts as a “bridge” for transferring H species to benzene adsorbed and activated at NiO site, which has significant implications to multifunctional nanocatalysts design for wide ranges of catalytic reactions.