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1.
Carmelo Gurnari Maria Teresa Voso Katia Girardi Angela Mastronuzzi Luisa Strocchio 《International journal of molecular sciences》2021,22(2)
Acute promyelocytic leukemia (APL) represents a paradigm of precision medicine. Indeed, in the last decades, the introduction of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) completely revolutionized the therapeutic approach to this previously highly fatal disorder. This entirely chemotherapy-free treatment, which provided excellent survival rates, has been initially validated in adults and, recently, translated in the pediatric setting. This review summarizes currently available data on the use of ATRA and ATO combination in pediatric APL, providing a particular focus on peculiar issues and challenges, such as the occurrence of pseudotumor cerebri and death during induction (early death), as well as the advantage offered by the ATO/ATRA combination in sparing long-term sequelae. 相似文献
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Targeted editing of intronic-splicing silencer enhancement of SMN2 Exon 7 inclusion by CRISPR/Case 9
LIUCHENG WU YI WANG LILI DU GUIQING JI RUI ZHOU ZEYI ZHAO JUN CHEN SHUNXING ZHU 《Biocell》2021,45(6):1501-1507
Spinal muscular atrophy (SMA) is an autosomal recessive hereditary neuromuscular disease. Exon 7 and 8 of survival of motor neuron 1 (SMN1) gene or only exon 7 homology deletion leads to the failure to produce a full-length SMN gene. The copy number of SMN2 gene with high homology of SMN1 affects the degree of disease and was the target gene for targeting therapy, in which splicing silencer in intron 7 was the key to suppress the inclusion of exon 7. In this study, we projected to use CRISPR/Case 9 for the targeted editing of intronic-splicing silencer (ISS) sequence to promote the inclusion of SMN2 exon 7 and increase the production of SMN2 full-length (FL) gene expression. It happens that there was a protospacer adjacent motif (PAM) at one end of the ISS sequence according to the design of sgRNA. The recombinant vector of sgRNA HSMN2 CRISPR/Case 9 was constructed and transfected into HEK293 cells. Sequencing results showed that the ISS sequence could be edited accurately and targeting in the predicted direction, in which deleting small fragments, inserting small amounts and mutation. Quantitative analysis of RT-PCR products by restriction enzyme of DdeI digestion showed that the FL of SMN2 increased by 8% (P < 0.05). In the primary cultured chondrocytes of SMA mice, in which sgRNA HSMN2 CRISPR/Case9 recombinant vector transfection could increase the SMN2 FL gene by 23% (P < 0.05) and significantly improve SMN protein levels (P < 0.05). CRISPR/Case 9 is an effective tool for gene editing and therapy of hereditary diseases, but it is rarely reported in the treatment of SMA diseases. This study shows that CRISPR/Case 9 was first used for the precision target of ISS sequence editing, which can effectively promote the production of SMN2 FL gene expressions, in which there was an important clinical reference value. 相似文献
4.
为了探讨氮含量及固溶温度对21-6-9不锈钢组织和硬度的影响,分别在950、1000、1050和1100 ℃对3种不同氮含量的热轧态21-6-9不锈钢进行1 h固溶处理,通过光学显微镜观察其组织结构,结合Thermo-Calc热力学计算对试验钢的微观组织进行分析,并对其进行硬度测试。结果表明,0.20%~0.28%N的21-6-9不锈钢热轧后沿轧制方向析出铁素体,且钢中铁素体经950~1100 ℃固溶处理可消除,当N含量达到0.34%时,试验钢中不再出现铁素体。随着固溶处理温度的升高,21-6-9不锈钢的晶粒组织长大,硬度降低。N含量的增加可显著提高固溶态21-6-9不锈钢的硬度,其增加程度随固溶处理温度的升高而减弱。 相似文献
5.
Daisuke Yamanaka Suzuka Kurita Yuka Hanayama Yoshiyuki Adachi 《International journal of molecular sciences》2021,22(4)
β-Glucan is widely distributed in various plants and microorganisms and is composed of β-1,3-linked d-glucose units. It may have a branched short or long side chain of glucose units with β-1,6- or β-1,4-linkage. Numerous studies have investigated different β-glucans and revealed their bioactivities. To understand the structure-function relationship of β-glucan, we constructed a split-luciferase complementation assay for the structural analysis of long-chain β-1,6-branched β-1,3-glucan. The N- and C-terminal fragments of luciferase from deep-sea shrimp were fused to insect-derived β-1,3-glucan recognition protein and fungal endo-β-1,6-glucanase (Neg1)-derived β-1,6-glucan recognition protein, respectively. In this approach, two β-glucan recognition proteins bound to β-glucan molecules come into close proximity, resulting in the assembly of the full-length reporter enzyme and induction of transient luciferase activity, indicative of the structure of β-glucan. To test the applicability of this assay, β-glucan and two β-glucan recognition proteins were mixed, resulting in an increase in the luminescence intensity in a β-1,3-glucan with a long polymer of β-1,6-glucan in a dose-dependent manner. This simple test also allows the monitoring of real-time changes in the side chain structure and serves as a convenient method to distinguish between β-1,3-glucan and long-chain β-1,6-branched β-1,3-glucan in various soluble and insoluble β-glucans. 相似文献
6.
The disintegration of waste active sludge was investigated by photo‐Fenton processes. A batch study was conducted to evaluate parameters, such as Fe2+ and Fe0 ions and H2O2, governing the activated sludge integration by the photo‐Fenton process. Under optimum conditions, the concentration of soluble chemical oxygen demand (SCOD) with the classical Fenton process (CFP) increased very rapidly in the first five minutes due to the sufficient presence of reaction components in the medium, and then the rate of increase declined. In the modified Fenton process (FTP), the SCOD concentration increased more slowly as metallic iron powder must first be dissolved. The photo‐Fenton process proved to be a feasible and efficient process for the disintegration of waste sludge. 相似文献
7.
采用X射线荧光分析仪、X射线衍射仪、N2物理吸附仪、场发射扫描电子显微镜等仪器表征了苏州、广西和美国佐治亚州3个产地的高岭土,并以此3种高岭土为原料制备了模型催化裂化(FCC)催化剂,在ACE评价装置上对比了模型催化剂的反应性能。结果表明:苏州及广西高岭土主要组分为高岭石,佐治亚高岭土主要组分为地开石及珍珠陶土;苏州高岭土呈片状,还含有少量棒状颗粒;广西高岭土呈多层片状,晶粒粒径较大;佐治亚高岭土呈薄片状,晶粒粒径较小;3种高岭土制备的模型催化剂反应活性、Na2O及RE2O3质量分数相近;广西高岭土制备的模型催化剂具有最大的孔体积和磨损指数,但比表面积最小,具有较强的重油转化能力,其目标产物(液化气+汽油)和副产物(干气+焦炭)收率都高于苏州、佐治亚高岭土制备的催化剂的。 相似文献
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9.
《Advanced Powder Technology》2022,33(3):103450
In the present investigation, systematic grinding experiments were conducted in a laboratory ball mill to determine the breakage properties of low-grade PGE bearing chromite ore. The population balance modeling technique was used to study the breakage parameters such as primary breakage distribution (Bi, j) and the specific rates of breakage (Si). The breakage and selection function values were determined for six feed sizes. The results stated that the breakage follows the first-order grinding kinetics for all the feed sizes. It was observed that the coarser feed sizes exhibit higher selection function values than the finer feed size. Further, an artificial neural network was used to predict breakage characteristics of low-grade PGE bearing chromite ore. The predicted results obtained from the neural network modeling were close to the experimental results with a correlation of determination R2 = 0.99 for both product size and selection function. 相似文献
10.
《International Journal of Hydrogen Energy》2022,47(79):33571-33596
This article provides a critical assessment of H2 from the standpoint of more widespread use as a sustainable fuel for Indian mobility applications in the global context. The potential techno-economic advantages of utilizing H2 for automobiles rather than battery electric vehicles or conventional internal combustion engine vehicles are emphasized. The present assessment demonstrates that H2 production, storage, and distribution costs are the primary challenges, and a significant improvement is still necessary for H2 to compete either against the internal combustion engine vehicle or the battery electric vehicle to win the race, arguably. The secondary challenges have also been demonstrated, which include the cost of the fuel cell stack and the modifications associated with internal combustion engine vehicles, as well as regulatory and safety concerns, which impede the widespread usage of H2. It is critical that policy-making for sustainable mobility in India is possible with the aid of a National H2 Energy Road-Map. This in turn can achieve a cost target of $0.5/kg for H2. 相似文献