An Engineered sgsh Mutant Zebrafish Recapitulates Molecular and Behavioural Pathobiology of Sanfilippo Syndrome A/MPS IIIA

Mucopolysaccharidosis IIIA (MPS IIIA, Sanfilippo syndrome type A), a paediatric neurological lysosomal storage disease, is caused by impaired function of the enzyme N-sulfoglucosamine sulfohydrolase (SGSH) resulting in impaired catabolism of heparan sulfate glycosaminoglycan (HS GAG) and its accumulation in tissues. MPS IIIA represents a significant proportion of childhood dementias. This condition generally leads to patient death in the teenage years, yet no effective therapy exists for MPS IIIA and a complete understanding of the mechanisms of MPS IIIA pathogenesis is lacking. Here, we employ targeted CRISPR/Cas9 mutagenesis to generate a model of MPS IIIA in the zebrafish, a model organism with strong genetic tractability and amenity for high-throughput screening. The sgsh^Δex5−6 zebrafish mutant exhibits a complete absence of Sgsh enzymatic activity, leading to progressive accumulation of HS degradation products with age. sgsh^Δex5−6 zebrafish faithfully recapitulate diverse CNS-specific features of MPS IIIA, including neuronal lysosomal overabundance, complex behavioural phenotypes, and profound, lifelong neuroinflammation. We further demonstrate that neuroinflammation in sgsh^Δex5−6 zebrafish is largely dependent on interleukin-1β and can be attenuated via the pharmacological inhibition of Caspase-1, which partially rescues behavioural abnormalities in sgsh^Δex5−6 mutant larvae in a context-dependent manner. We expect the sgsh^Δex5−6 zebrafish mutant to be a valuable resource in gaining a better understanding of MPS IIIA pathobiology towards the development of timely and effective therapeutic interventions.     

1,4-丁炔二醇加氢制1,4-丁烯二醇工艺及催化剂研究进展

论述1,4-丁烯二醇发展现状,简述1,4-丁烯二醇BYD加氢法、3,4-环氧-1-丁烯水解法和1,3-丁二烯法3种生产工艺特点.其中,BYD催化加氢法是生产BED技术最为成熟、应用最为广泛的生产工艺.BYD加氢反应是一个多步复杂反应.论述了BYD加氢催化反应过程以及3种催化剂(贵金属催化剂、过渡金属催化剂、骨架型Raney-Ni催化剂)在BYD加氢反应过程中的应用特点,指出Raney-Ni催化剂具有价格低、活性高的优点,开发中压下BYD加氢制备BED的Raney-Ni催化剂具有重要意义.     

纯铜动态再结晶的Monte Carlo法模拟

本文建立了一种基于能量判据的确定动态再结晶各个阶段临界应变的方法，并基于此种方法Monte Carlo(MC)法进行了纯铜动态再结晶的二维模拟，定量计算了变形引起的存储能与变形之间的关系，计算出了发生动态再结晶的临界应变。模拟结果表明，采用MC法可以揭示动态再结晶的形核生长过程与应变历史之间的关系。结合本文所建立的基于能量判据的确定动态再结晶各个阶段临界应变的方法，可以预测开始发生动态再结晶的临界变形条件。     

THE VAPOR PRESSURES OF ANTIMONY IN LIQUID CALLIUM-ANTIMONY ALLOYSS

采用气流输运法测定了720,750及780℃时液态纯Sb及Ga-Sb合金中组分Sb的蒸气压。由蒸气压的实验数据和Ga-Sb系的T-X相图构成了Ga-Sb系中Sb蒸气压的P-T图。     

线阵电荷耦合器技术在自动测长系统中的应用

介绍用线阵电荷耦合器件(CCD)技术设计的型材在线实时测量系统。采用粗测和精测相结合的方法，解决了较大长度范围内精确测量问题。详细介绍动态在线非接触式测控系统的基本工作原理及软硬件组成。实测结果表明其测量精度高、效果好，能满足快速流水线的现场检测。     

普通快走丝线切割机间隙电压取样电路浅析

介绍了传统的间隙电压取样电路，指出了它存在的问题，简述了取样电路的发展方向。     

Finite-element simulation of induction heat treatment

An efficient finite-element procedure has been developed for the analysis of induction heat treatment problems involving nonisothermal phase changes. The finite-element procedure first simulates the magnetic field developed when currents flow through an induction coil by solving Maxwell’s electromagnetic field equations; at the following step, it calculates the temperature distribution in the workpiece due to eddy currents induced by the magnetic field. The final stage of the simulation involves the determination of the distributions of residual stress, hardness, and microstructure in the workpiece. The finite-element analysis includes temperature-dependent material properties, changes in permeability of the workpiece at the Curie temperature, a mixed hardening rule to describe the material constitutive model, and the incorporation of time-temperature-transformation (TTT) diagram. The procedure was applied to the simulation of the induction hardening of 1080 steel bar. Firstly, the magnetic field and temperatures developed in the workpiece during (a) the induction heating of an infinitely long 1080 steel cylinder by a single encircling coil and (b) the induction heating of a semi-infinite half-space by a single coil suspended above it were calculated using the finite-element procedures. These were validated by comparing them with analytical solutions derived for these configurations using a Green’s function method. Finally, to demonstrate the predictive capability and practical applicability of the current finite-element procedure, two examples pertaining to the induction heat treatment of an infinite 1080 steel bar of square cross section and a notched finite 1080 steel cylinder of circular cross section were analyzed to predict the magnetic field, temperature, and residual stress distributions. The current finite-element procedure could be used as a powerful design tool for linking induction heat treating parameters with the mechanical property attributes of the heat treated component.     

火焰原子吸收光谱法测定电缆绝缘护套中的铅

提出了用空气—乙炔原子吸收光谱法测定电缆绝缘护套中的铅含量。铅在0.00~11.00μg.ml-1(r=0.9991)范围内有良好的线性关系。本法的RSD≤3.17%,回收率在97.2%~103.2%之间。     

The mechanical property and phase structures of wheat proteins/polyvinyl alcohol blends studied by high-resolution solid-state NMR

The phase structures of thermally processed wheat proteins (WP) and polyvinyl alcohol (PVOH) blends were studied by solid-state high-resolution NMR spectroscopy. The intermolecular interactions among the multi-component systems and the behavior of each component in the blends on scales of nanometers were examined. The mechanical properties of the blends were also measured and related to the phase structure studies. The results indicated that the polymer chains of WP could be homogeneously mobilized when thermally processed with glycerol and water as plasticisers, but the glycerol predominately associated with WP rather than PVOH in the blends. The intermolecular hydrogen bonding interactions between WP and PVOH caused some extent of miscibility in the system on scales of nanometers especially when the PVOH content was low. The tensile strength and modulus of the blends were improved as compared to WP. However, the intermolecular interactions were relatively weak and could not be further enhanced by increasing PVOH component in the blends. The particle miscible WP/PVOH blends contained plasticised WP and PVOH phases in conjunction with the miscible WP/PVOH phase. Increasing the PVOH content in the blends did not result in an increase of the percentage of the miscible phase and the blends tented to be immiscible while the elongation of the blends was reduced when increasing the PVOH content in the blends.     

区域烧结法制备高温气体清洁用大尺寸陶瓷过滤管

用区域烧结在1 d内可完成高温气体清洁用1.5 m陶瓷过滤管的制备过程,区域烧结的优势是缩短研究和开发周期.目前,经过认真设计,已经完成区域烧结设备.多孔陶瓷件比致密陶瓷件更容易烧结成型,原因是多孔陶瓷在区域烧结期间可免除其收缩和变形.