A Structured Ultramicroporous Metal-Organic Framework for Carbon Dioxide Capture

Comprehensive Summary Carbon dioxide(CO_(2))capture is one of the most important aspects of reducing global warming.In terms of CO_(2)capture,metal-organic frameworks(MOFs)have several advantages.However,it isn't easy to shape MOFs while maintaining their performance.Herein,we describe the development of a pellet-shaped ultramicroporous MOF,Ni(3-ain)2(3-ain=3-aminoinoisonicotinic acid),that is capable of selectively adsorbing CO_(2).Polyvinyl butyral(PVB)is used as a binder during the production of Ni(3-ain)2 MOF pellets.The adequately shaped material can maintain its crystallinity and exhibit a high CO_(2)adsorption capacity(3.73 mmol·g^(-1))at ambient conditions,which is significantly greater than those obtained for N_(2)(0.63 mmol·g^(-1))and CO(0.90 mmol·g^(-1)).Consequently,this material displays high IAST selectivities for CO_(2)/N_(2)(26.3,15/85,V/V)and CO_(2)/CO(19.2,1/99,V/V).According to the theoretical calculations,Ni(3-ain)2 preferentially adsorbs CO_(2)molecules over N_(2)molecules and CO molecules.The results of experiments on dynamic breakthrough have demonstrated that Ni(3-ain)2 pellets are capable of effectively separating CO_(2)/N_(2)or CO_(2)/CO mixtures under conditions of dynamic flow.Furthermore,the structured MOF materials can be synthesized in one step at kilogram scale.This work provides an avenue for the shaping of MOFs for potential industrial applications in the future.

一个丙酮酸激酶缺乏症家系PKLR基因的突变分析及产前诊断

目的对一个丙酮酸激酶缺乏症（pyruvatekinasedeficiency，PKD）家系进行致病基因突变分析及产前诊断。方法应用目标序列捕获和高通量测序技术对临床拟诊为PKD的患儿的PKLR基因外显子及其侧翼序列进行测序，采用SIFT及PolyPhen-2数据库对突变进行蛋白功能预测，在确定先证者致病基因型后，应用Sanger测序技术进行验证，同时检测其父母基因型，并对孕16周的高危胎儿抽取羊水进行产前诊断。结果患儿PKLR基因存在罕见的双重杂合突变C．661G〉A（Asp221Asn）以及C．1528C〉T（Arg510Ter），导致该基因的第221位氨基酸由天冬氨酸变化为天冬酰胺，第510位由精氨酸改变为终止密码子。Sanger测序验证了该双重突变的存在，先证者父母分别检出c．661G〉A（Asp221Asn）与c．1528C〉T（Arg5lOTer）突变。胎儿检出与先证者相同的致病突变。在终止妊娠后，对流产物进行突变位点分析，结果与产前诊断一致。结论PKLR基因c．661G〉A与C．1528C〉T复合突变是该PKD家系的分子发病机制。通过产前诊断可以有效阻止致病基因的传递，降低生育患儿的风险。