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.     

磷石膏提质降杂实验研究

磷石膏的综合利用对保护环境、促进企业可持续健康发展具有重要意义,但是磷石膏中的磷、氟、二氧化硅等杂质影响其利用。针对云南磷石膏中二氧化硅含量高的问题,采用正浮选工艺开展了磷石膏提质降杂的实验研究。通过单因素条件实验获得了合适的工艺参数：浮选温度为15 ℃、矿浆pH为2、十二胺用量为300 g/t。在此条件下,进行了“一粗二精”的正浮选开路流程实验,获得了二水石膏（CaSO₄·2H₂O）质量分数为97.5%、二氧化硅质量分数为1.17%的精矿,所得精矿达到了GB/T 23456—2018《磷石膏》一级品指标的要求;二水石膏回收率为98.58%、二氧化硅去除率为92.23%。通过正浮选不仅成功实现了二水石膏和二氧化硅的有效分离,而且磷石膏中的水溶磷和水溶氟也得到了有效去除,从而提高了磷石膏的品质。     

微张力减径机轧后钢管产生青线的原因分析

分析出某厂14机架单独传动钢管微张力减径机在轧制过程中出现青线的原因是该减径机的主机架中滑板制造误差过大，机架受力倾斜造成的，整改后青线消失。     

Initial stages of tin electrodeposition from sulfate baths in the presence of gluconate

Tin electrodeposition in its initial stages in acid sulfate/gluconate baths was studied with varying tin and gluconate concentrations using potential-controlled electrochemical techniques. The deposit morphology was observed by scanning electron microscopy (SEM). A comparison with tin electrodeposition from acid sulfate baths in the absence of gluconate was also carried out. Use of a highly acidic bath leads to nonuniform deposits, even in the presence of gluconate; at pH 4 deposits are uniform, brilliant and suitable for finishing applications. Tin crystallites have a well defined morphology which depends on bath agitation conditions. In the absence of agitation, the crystallites have the same tetragonal shape as in a sulfate bath without gluconate.     

关于生产精制元明粉的探讨

介绍了以山西运城南风自产芒硝为原料生产精制元明粉的几种工艺,以及国内外精制元明粉的市场及生产状况.精制工艺包括化学除杂法、原料优选法、重结晶法.结果表明,精制元明粉的生产过程实质是一个除杂、提纯过程：对于钙离子含量高的原料,可采用化学除杂法;对于氯离子含量高的原料,可采用氯离子富集法、冷冻法或洗涤法;镁离子较难去除,可采用水硝洗涤、冷冻法.     

Guayule seedling response to nitrogen fertilizers

Slow growth and high seedling mortality limit direct seeding establishment of guayule (Parthenium argentatum G.). This study was conducted to assess seedling growth enhancement by the addition of different rates and forms of N fertilizers and Ca salts. Experiments were conducted in a greenhouse with cultivar 593 under both surface and subirrigated conditions using water low in salts (salinity of 0.8 dSm^–1, SAR of 5.0 and 10 mg Ca L^–1). Under surface-irrigated conditions, seedling height and fresh plant weight increased with N application to the irrigation water to 70 mg L^–1. The best seedling growth was observed when (NH₄)₂SO₄ was added in combination with CaCl₂ or CaSO₄. Progressively less growth was observed by addition of (NH₄)₂SO₄ alone, CO(NH₂)₂ plus CaSO₄, CO(NH₂)₂ alone and Ca(NO₃)₂. When seedlings were subirrigated, however, the best growth was observed with Ca(NO₃)₂. Intermediate growth was obtained with (NH₄)₂SO₄ plus CaSO₄ and lowest growth rates with (NH₄)₂SO₄ alone. These differential responses may be explained by the differences in leaching and volatile characteristics of the N forms. Growth enhancement from N and Ca additions increased with time with significant increases 45 days after seeding. Nitrogen application with Ca may be effective amendment in promoting subsequent growth of direct seeded guayule.     

硫酸铜灭藻影响因素的试验研究

目的通过对硫酸铜灭藻影响因素的试验研究,确定出硫酸铜灭藻的最佳工况条件.方法以叶绿素a法作为检测方法,通过烧杯试验,确定处理含藻水的最佳硫酸铜投加量、接触反应时间、pH等参数.结果硫酸铜最佳投加量为1.5 mg/L;硫酸铜适宜的接触反应时间为8～12 h;pH值6.6左右时,硫酸铜藻类去除率较高,pH值＞9时,藻类去除率显著降低.结论 pH值对硫酸铜灭藻效果的影响较大;温度对硫酸铜灭藻有一定的影响;水中还原物质浓度对硫酸铜灭藻影响较小;藻类浓度不同对硫酸铜灭藻效果有一定的影响.     

快速法测定AES中未硫酸化物的含量

讨论了一种快速测量AES中未硫酸化物的方法。首先将配制好的AES-乙醇溶液，通过离子交换树脂，然后收集、蒸馏、干燥得到的液体，称重后即可得到未硫酸化物的含量，采用该方法比国家标准节约2．5～3h的分析时间。     

盐水中硫酸根的脱除技术

指出了盐水中硫酸根的危害，介绍了盐水生产中脱除硫酸根的主要技术方法。     

燃烧固硫反应的动力学和热力学及其影响固硫产物分解因素的研究

煤在高温条件下燃烧不利于固硫反应，并从热力学角度出发，得出了煤燃烧中钙基固硫剂反应自由能与温度的关系函数，由化学反应动力学得出院 硫酸钙分解反应的动力学方程及其相关参数，在还原性气氛中，从理论和实验上都证明了硫酸钙在较低温下会发生二次分解，理论分解温度与实际分解温度非常吻合，煤灰中氧化钙和三氧化二铝的存在，可以提高硫酸钙的分解温度同阳也给出了钙基固硫剂的应用条件。