Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder resulting from motor neuron death. Approximately 10% of cases are familial (FALS), typically with a dominant inheritance mode. Despite numerous advances in recent years, nearly 50% of FALS cases have unknown genetic aetiology. Here we show that mutations within the profilin 1 (PFN1) gene can cause FALS. PFN1 is crucial for the conversion of monomeric (G)-actin to filamentous (F)-actin. Exome sequencing of two large ALS families showed different mutations within the PFN1 gene. Further sequence analysis identified 4 mutations in 7 out of 274 FALS cases. Cells expressing PFN1 mutants contain ubiquitinated, insoluble aggregates that in many cases contain the ALS-associated protein TDP-43. PFN1 mutants also display decreased bound actin levels and can inhibit axon outgrowth. Furthermore, primary motor neurons expressing mutant PFN1 display smaller growth cones with a reduced F/G-actin ratio. These observations further document that cytoskeletal pathway alterations contribute to ALS pathogenesis.     

Fabrication and sintering behavior of high-nitrogen nickel-free stainless steels by metal injection molding

High-nitrogen nickel-free stainless steels were fabricated by the metal injection molding technique using high nitrogen alloying powders and a mixture of three polymers as binders. Mixtures of metal powders and binders with various proportions were also investigated, and an optimum powder loading capacity was determined as 64vol%. Intact injection molded compacts were successfully obtained by regulating the processing parameters. The debinding process for molded compacts was optimized with a combination of thermo-gravimetric analysis and differential scanning calorimetry analysis. An optimum relative density and nitrogen content of the specimens are obtained at 1360℃, which are 97.8% and 0.79wt%, respectively.     

Modeling the austenite-ferrite transformation in microalloyed steel P510L

Based on experimental results, the transformation kinetics and cooling characteristics of low-carbon steel were analyzed and modeled to quantitatively link the operational parameters of a process with the properties. From the continuous cooling transformation results, comparisons of the start temperature of austenite-ferrite transformation among three models were analyzed, and the optimal lnk and n, which are the parameters in the Avrami equation, were determined by applying two regression models at different cooling rates. The transformation kinetics during continuous cooling was determined. Furthermore, reasonable agreements between experimental results and predictions were obtained, which can demonstrate the rationality of the established models.     

Structure,optical, and magnetic properties of Mn-doped ZnO films prepared by sputtering

The microstructural, optical, and magnetic properties and room-temperature photoluminescence (PL) of Mn-doped ZnO thin films were studied. The chemical compositions were examined by energy dispersive X-ray spectroscopy (EDS) and the charge state of Mn ions in the ZnO:Mn films was characterized by X-ray photoelectronic spectrometry (XPS). From the X-ray diffraction (XRD) data of the samples, it can be found that Mn doping does not change the orientation of ZnO thin films. All the films prepared have a wurtzite structure and grow mainly along the c-axis orientation. The grain size and the residual stress were calculated from the XRD results. The optical transmittance of the film decreases with the increase of manganese content in ZnO. The room-temperature photoluminescence of the films shows that the intensity of near band energy (NBE) emission depends strongly on the Mn content. The hysteresis behavior indicates that the films with the Mn content below 9at% are ferromagnetic at room temperature.     

Electrochemical corrosion failure mechanism of M152 steel under a salt-spray environment

The corrosion failure mechanism of M152 was studied using the neutral salt-spray test to better understand the corrosion behavior of 1Cr12Ni3Mo2VN (M152), provide a basis for the optimization of material selection, and prevent the occurrence of failure. Moreover, the mechanism was investigated using the mass loss method, polarization curves, electrochemical impedance spectroscopy (EIS), stereology microscopy, scanning electron microscopy, and energy-dispersive X-ray spectroscopy (EDS). The results show that M152 steel suffers severe corrosion, especially pitting corrosion, in a high-salt-spray environment. In the early stage of the experiment, the color of the corrosion products was mainly orange. The products then gradually evolved into a dense, brown substance, which coincided with a decrease of corrosion rate. Correspondingly, the EIS spectrum of M152 in the late test also exhibited three time constants and presented Warburg impedance at low frequencies.     

Leaching of the residue from the dry off-gas de-dusting and desulfurization process of an iron ore sinter plant

The residue from a second-stage dry sinter plant off-gas cleaning process contains both the fine dust from the sinter plant and the sorbent used. Recycling of the material that is usually handled by landfills to the sinter plant feed is not possible because of its chloride content. Leaching of the chlorides allow the recycling of remaining solids. The saline leachate produced contains some heavy metals and must be treated before it is discharged into the sea. In laboratory experiments, leaching tests with the subsequent treatment of the leachate were conducted. After the process was optimized, all heavy-metal concentrations were below the permissible values. The optimum treatment conditions for heavy-metal precipitation were observed to be the filtration of the suspended solids followed by the dosing of liquid with lime milk (pH 10) and the subsequent precipitation using sodium sulfide.     

Self-organized ordering of earthquakes(M≥8) in Mainland China

<正>The paper presented a new regular pattern(network structure) of great earthquakes occurred in Mainland China during the past 700 years,which may be helpful to improve the understanding of great earthquakes and can serve as a base for the study of prediction of future great earthquakes.It can be done because there are quite complete and confident records of historical and recent earthquakes in a wide extent in China.     

Experimental Study of Fluid Flow in Thin Slab Continuous Caster Mould with Water-Model

Model study is an efficient method for optimizing the siructure of the mould and the submerged entry nozzle (SEN). Based on the similarity criteria, a full-scale water model has been established in accordance with the mould of thin-slab caster of the CSP (Compact Strip Production) operation. The effects of SEN structure including outlet area, outflow angle, nozzle width, thick-ness and immersion depth have been studied under high speed casting by measuring the amplitude and the impetus of top waves. By the orthogonal experiment design, not only the influence of the factors was estimated, but also the optimum work condition was judged. The rules of the fluid flow phenomena were summarized. The principle for choosing a reasonable structure of SEN was discussed.     

Development of a New Subermerged Entry Nozzle in Thin Slab Caster

The fluid flow in the mould of the thin slab continuous caster has a large influence on the quality of slabs and its productivity.The fluid flow pattern can be controlled by the SEN (Submerged Entry Nozzle) structure. Traditional SEN can not decrease the surface turbulence and penetration depth at the same time, especially at high casting speed. In order to improve the fluid flow in the mould, a new structure SEN-Dissipation SEN have been invented. The water modeling experiments proved that the dissipation SEN could satisfy, the needs of fluid flow condition in the mould at high casting speed.     

Underground Pipeline Surveillance with an Algorithm Based on Statistical Time-Frequency Acoustic Features

Underground pipeline networks suffer from severe damage by earth-moving devices due to rapid urbanization. Thus, designing a round-the-clock intelligent surveillance system has become crucial and urgent. In this study, we develop an acoustic signal-based excavation device recognition system for underground pipeline protection.The front-end hardware system is equipped with an acoustic sensor array, an Analog-to-Digital Converter(ADC)module(ADS1274), and an industrial processor Advanced RISC Machi...