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| 1 | Base editing-mediated splicing correction therapy for spinal muscular atrophy显示文摘Dear Editor,Spinal muscular atrophy(SMA)is a devastating autosomal recessive motor neuron disease.1,2 Infants with more severe forms of type I SMA die before the age of 2 if no intervention is provided.1,2 Spinraza and zolgensma have been approved by the FDA as SMA therapeutics for pediatric patients.1,2 However,as an antisense oligonucleotide(ASO)based therapy,spinraza requires four loading doses,followed by three annual maintenance doses.The patients would be subjected to repeated intrathecal injections in the procedure.Zolgensma is a single-dose gene-replacement therapy for SMA,but unfortunately is unreliable in maintaining a high,stable level of gene expression.These issues have limited the therapeutic effects of the two approved drugs for SMA. | Xiang Lin Haizhu Chen Ying-Qian Lu Shunyan Hong Xinde Hu Yanxia Gao Lu-Lu Lai Jin-Jing Li Zishuai Wang Wenqin Ying Lixiang Ma Ning Wang Erwei Zuo Hui Yang Wan-Jin Chen | 2020 | Cell Research2020，30，6： | 5 |
| 2 | Purification of scandium from concentrate generated from titanium pigments production waste显示文摘Nowadays 80%of scandium in China is obtained from titanium pigments production waste through a complex purification process.The study mainly focused on the purification of Sc from its concentrate generated from titanium pigments production waste by solvent extraction.Several extractants have been tried and 10%D_(2)EHPA-5%TBP-85%sulfonated kerosene exhibited the best extraction performance towards Sc in 7 mol/L H_(2)SO_(4)solution,so it was selected as the oil phase.0.5%of H_(2)O_(2)was added into the concentrated solution which can effectively inhibit the extraction of Ti.Both the extraction and back extraction parameters are optimized.The preferred extraction conditions were obtained,i.e.,acidity:7 mol/L H_(2)SO_(4),the phase ratio A/O:10,room temperature,mixed contact time:30 min,Sc concentrate:10 g/L,that the extraction rate of Sc in the above conditions was nearly 100%.NaOH was used for back extraction with the stripping rate 99%on the following conditions:5 mol/L NaOH stripping for 30 min at a phase ratio A/O:1 at 90℃.Finally,H_(2)C_(2)O_(4)was used to further purify the back extraction product and Sc_(2)(C_(2)O_(4))_(3)precipitant fo rmed.The final product Sc_(2)O_(3)with a purity over 99.5%was obtained by calcining Sc_(2)(C_(2)O_(4))_(3)at 1000℃for 2 h.A conceptual process for Sc purification was put forward and proved.The total recovery yield of Sc in the whole process is 95%. | Jie Zhou Shunyan Ning Jiejie Meng Shichang Zhang Wei Zhang Siyi Wang Yedan Chen Xinpeng Wang Yuezhou Wei | 2021 | Journal of Rare Earths2021，39，2： | 4 |
| 3 | Stability of isoHex-BTP/SiO_2-P adsorbent against acidic hydrolysis and γ-irradiation显示文摘Separation of trivalent minor actinides(MA(ⅡI): Am(ⅡI), Cm(ⅡI)) from fission products(FP) in high-level liquid waste(HLLW) is an important task in advanced nuclear-fuel reprocessing systems. For this purpose, an advanced aqueous partitioning process based on extraction chromatography method was studied. Because R-BTP extractants(R-BTP: 2,6-bis(5,6-dialkyl-1,2,4-triazin-3-yl)pyridine, R = alkyl group) exhibit high selectivity for MA(ⅡI) over trivalent rare-earth elements(RE(ⅡI)), a novel adsorbent isoHex-BTP/SiO2-P was prepared by impregnating isoHex-BTP extractant into the macroporous SiO2-P support with a mean diameter of 60 μm. The stability of isoHex-BTP/SiO2-P against nitric acid and γ-irradiation was investigated. It was found that isoHex-BTP/SiO2-P adsorbent shows good adsorption affinity to Dy(ⅡI). The hydrolytic and radiolytic stability of isoHex-BTP/SiO2-P adsorbent in 0.01 mol/L HNO3 was fairly promising. However, the adsorption amount Q of Dy(ⅡI) decreased dramatically in 3 mol/L HNO3 with the increase of the absorbed dose and became nearly zero at the absorbed dose over 46 kGy. These results suggest that with the synergetic effect of radiation and acidic hydrolysis, the adsorbent instantly loses its efficacy. | WANG XinPeng NING ShunYan LIU RuiQin WEI YueZhou | 2014 | Science China Chemistry2014，57，11： | 1 |
| 4 | Adsorption behavior of Me2-CA-BTP/SiO2-P adsorbent toward MA(Ⅲ) and Ln(Ⅲ) in nitrate solution显示文摘A porous Me_2-CA-BTP/SiO_2-P adsorbent was prepared to separate MA(Ⅲ) from Ln(Ⅲ) in high level liquid waste(HLLW).The adsorption behavior of Me_2-CA-BTP/SiO_2-P toward ^(241)Am(Ⅲ) and Ln(Ⅲ) in 0.01 M HNO_3-NaNO_3 solution was studied.Me_2-CA-BTP/SiO_2-P showed high adsorption and selectivity toward ^(241)Am(Ⅲ) over Ln(Ⅲ) fission products with the separation factor(SF) reaching to 557,2355,1952,1082,214,105,86,14 for Y,La,Ce,Nd,Sm,Eu,Gd and Dy respectively in 0.01 M HNO_3-0.99 M NaNO_3 solution.The adsorption kinetics of both Dy(Ⅲ) and Eu(Ⅲ) on Me_2-CA-BTP/SiO_2-P was studied and followed pseudo-second-order rate equation indicating chemical sorption as the rate-limiting step of the adsorption,and the adsorption isotherm of Dy(Ⅲ) and Eu(Ⅲ) matched better with the Langmuir isotherm than the Freundlich isotherm with the adsorption amount around 0.22 and 0.20 mmol/g respectively.Thermodynamic study revealed that the adsorption of both Dy(Ⅲ)and Eu(Ⅲ) on Me_2-CA-BTP/SiO_2-P was spontaneous and endothermic processes with a positive entropy at 298,308,313 K. | Shunyan Ning Qing Zou XinpengWang RuiqinLiu Yuezhou Wei | 2016 | Science China Chemistry2016，59，7： | 1 |
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