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91.
Alon M. Douek Mitra Amiri Khabooshan Jason Henry Sebastian-Alexander Stamatis Florian Kreuder Georg Ramm Minna-Liisa nk Donald Wlodkowic Jan Kaslin 《International journal of molecular sciences》2021,22(11)
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 sgshΔex5−6 zebrafish mutant exhibits a complete absence of Sgsh enzymatic activity, leading to progressive accumulation of HS degradation products with age. sgshΔex5−6 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 sgshΔex5−6 zebrafish is largely dependent on interleukin-1β and can be attenuated via the pharmacological inhibition of Caspase-1, which partially rescues behavioural abnormalities in sgshΔex5−6 mutant larvae in a context-dependent manner. We expect the sgshΔex5−6 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. 相似文献
92.
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. 相似文献
93.
94.
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。 相似文献
95.
96.
97.
Finite-element simulation of induction heat treatment 总被引:2,自引:0,他引:2
K. F. Wang S. Chandrasekar Henry T. Y. Yang 《Journal of Materials Engineering and Performance》1992,1(1):97-112
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. 相似文献
98.
99.
The mechanical property and phase structures of wheat proteins/polyvinyl alcohol blends studied by high-resolution solid-state NMR 总被引:1,自引:0,他引:1
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. 相似文献
100.