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Niemann-Pick type C (NPC) disease is a fatal inherited neurodegenerative disorder caused by loss-of-function mutations in the NPC1or NPC2gene. There is no effective way to treat NPC disease. In this study, we used adeno-associated virus (AAV) serotype 9 (AAV9) to deliver a functional NPC1gene systemically into NPC1−/−mice at postnatal day 4. One single AAV9-NPC1 injection resulted in robust NPC1 expression in various tissues, including brain, heart, and lung. Strikingly, AAV9-mediated NPC1 delivery significantly promoted Purkinje cell survival, restored locomotor activity and coordination, and increased the lifespan of NPC1−/−mice. Our work suggests that AAV-based gene therapy is a promising means to treat NPC disease.

An optimization was performed for a sintering waste heat power unit with all data obtained in the site and under the unit normal operating conditions. The physical and mathematical model for the process of cooling and generation is established, which makes the net power generation as an objective function of the cooling machine imported ventilation, the thickness of sinter and the main steam pressure. Optimizing for single parameter, we found that each parameter had an optimal value for the system. In order to further optimize the system's operating parameters, genetic algorithm was used to make the combinatorial optimization of the three parameters. Optimization results show that power generation capacity per ton is increased by13.10%, and net power generation is increased by 16.17%. The optimization is instructive to the operation of sintering waste heat power unit.

Using ANSYS finite element analysis software, the crushing effect and wall rock deformation of a small high-pressure jet impacting coal were given numerical simulation analysis. It verified that impacting increased exposed area of coal in punch, providing conditions for pressure relief of internal coal seam and gas seepage. through comprehensive analysis of the rock stress and coal displacement of different roadway models after undercutting, it was determined that aperture size was 4/15 or so of roadway floor length, thus roadway fissures developed full, and maintained rock integrity, in favor of speeding up the face driving.

Through ANSYS/LS-DYNA simulates mini-jet fracturing coal effect, this paper verifies that high-pressure mini-jet forms ahead hole on working face coal, which increases coal exposing area and relieves pressure inside the coal bed to creative conditions for gas relieving and flowing. Additionally underground stress, the coal around hole shifts to the hole space and enlarges the hole relieving pressure and gas exhausting scope further, which removes the coal outburst potential within the control scope and provides theoretical basis for studing the equipment of high-pressure mini-jet cutting hole against outburst.

Soft rock roadway is one challenge of roadway support. With plastic state, it has a certain bearing strength, and its large plastic performance must release out in some form. Therefore soft rock roadway is not a single support effective, it must use a joint way. By numerical simulation, this paper analyzes uniform diffusion of bolt mesh support to wall rock stress, which provides a theoretical basis for bolt mesh support of soft rock.

Through the synthetically analysis of the characteristics of coal mining and the environment quality of the mining area, the environment quality evaluation index system is formed: water and soil environment quality are the principal layer, the influence factors are measure layer. The evaluation degree of every factor is defined by fuzzy synthesis evaluation method and the environment quality level and the problem urgently to result are found, which provides theory support for environmental protection and green development.

As impacting and cutting role of the small jet, coal around slots occured violent displacement and expansion, increasing coal cracks, which greatly improved gas flow in coal seams and made for gas emission. The original coal stress changed and stress concentration belt moved to coal seams depth constantly. With gradual relief expansion and fracture increased, coal seam permeability coefficient increased, gas pressure decreased. Coal potentiality released, coal crushed and moved, a lot of gas with adsorption state changed free, then gas fully released and gas pressure gradient decreased, which reduced outstanding risk. it was determined that aperture size was 4/15 or so of roadway floor length using ANSYS finite element analysis software, thus roadway fissures developed full, and maintained rock integrity, in favor of speeding up the face driving.

Fire hazard is one of the severe casualty accident, which injures the life-safety of the miner crucially and disturbs the sustained development of the coal mine. Recently the check-up table is the mere method applying for the assessment of the fire hazard, and the assessment conclusion is subjective intensively and the Index System of the Safety Assessment is not perfect, because the dangerous degree of fire hazard is set up by the professional. In this paper, the seven factors were generalized through the statistic and analysis of the 56 fire hazards, which included self-ignite gradation of coal bed, miner stuff, management of ventilation of coal pits, fire control system, safety administration, mine combustible, risk ratio of the electrical equipment. The ratio of each factor was confirmed by the method of layered analysis, the safety degree was set up through data processing and the proper measure and suggestion were put forward according to the safety result.

Through the synthetically analysis of the composite index method for evaluation of rock burst rating, the limitation of this method is discussed. Mines have various burst development characteristics, and geological condition and mining technical factors decide different rock burst weights greatly. So this paper prefers an optimal case based on analytic hierarchy process, using cooperation matrix to decide the influence weight of the factors on the burst, and synthetized with the actual impact risk index, which makes the method more precise and objective to evaluate the burst rating to provide a scientific basis for safe investment of the mine.

With the development of technology and the society, the demand standard of enterprise to talented person is more and more strict. Through analyzing the special function of some methods, such as to carry out tutorial system, to reform teaching methods, to strengthen teaching management, to improve engineering test methods and improve school infrastructure measures, this paper discusses the new method to cultivate high quality engineering and technical personnel to provid theoretical guidance for future universities to meet market demand and cultivate innovative talents.