摘要：The rational design and construction of inexpensive and highly active electrocatalysts for hydrogen evolution reaction(HER)is of great importance for water ***,we develop a facile approach for preparation of porous carbon-confined Ru-doped Cu nanoparticles(denoted as Ru-Cu@C)by direct pyrolysis of the Ru-exchanged Cu-BTC metal–organic *** served as the electrocatalyst for HER,strikingly,the obtained Ru-Cu@C catalyst exhibits an ultralow overpotential(only 20 mV at 10 mA cm^(-2))with a small Tafel slope of 37 m V dec^(-1)in alkaline *** excellent performance is comparable or even superior to that of commercial Pt/C *** functional theory(DFT)calculations confirm that introducing Ru atoms into Cu nanocrystals can significantly alter the desorption of H_(2) to achieve a close-to-zero hydrogen adsorption energy and thereby boost the HER *** strategy gives a fresh impetus to explore low-cost and high-performance catalysts for HER in alkaline media.

摘要：炭基肥料是以生物炭为基质与其他肥料复合而成的新型肥料,能有效提高土壤的肥力和透气能力,同时具有对肥料的缓释效果等。为了炭基肥料成型工艺优化及工业化生产,该文以复混肥料的国家标准为参考,尿素、过磷酸钙、磷酸氢二铵、氯化钾作为提供氮磷钾元素的基础肥料,并以秸秆炭为基质,着重讨论了成型前加水量、有无添加胶黏剂和成型前物料粒度对炭基肥料成型的影响。研究成型炭基肥料的较佳工艺为基础肥料质量分数占70.36%,总养分的质量分数为28%,其中N:P2O5:K2O养分质量比为10:8:10,秸秆炭质量分数16.64%,不加研磨,配以13%的水,搅拌均匀常温造粒成型;并对在此条件下成型后的炭基肥料养分含量、含水率、溶水性、强度、pH值和对土壤化学性质的影响等性能进行表征,指标如下:氮质量分数为10.07%,水溶性磷质量分数5.47%,有效磷质量分数8.38%,水溶性磷占有效磷65.27%,钾质量分数10.45%,水分质量分数3.24%,机械强度85%以上,pH值为6.41。实测结果符合国家相应标准,工艺设计简单可行,进而为炭基肥料的工业生产提供参考。

摘要：目的总结1例COL1A2新突变致胎儿严重成骨不全症（OI）的临床特征及基因突变的特点,为胎儿产前咨询提供依据。方法对产检B超检查示OI可能的胎儿流产组织抽提DNA进行基因型分析,自行设计COL1A1和COL1A2所有外显子及剪接区域的引物。利用Sanger测序法对胎儿行COL1A1和COL1A2基因外显子及剪接区域的测序分析并行父母验证。依据人类基因突变数据库（HGMD）专业版,对COL1A2突变所致疾病临床表型行文献复习。结果胎儿的COL1A1和COL1A2基因均检测出变异位点。COL1A2基因检测到杂合突变（c.3142G〉T,***1048Cys）在寡核苷酸多态性数据库、HGMD及Ⅰ型胶原蛋白突变数据库均未见报道,结合胎儿父母验证为新发突变,对比公共数据库及在线预测软件预测该突变类型为致病突变。在HGMD专业版中搜索COL1A2,共检索到387个COL1A2致病突变,与21种疾病及其亚型相关。92%的突变引起OI或其亚型,还可引起Ehlers-Danlos综合征或其亚型。结合COL1A2突变所致疾病临床表型行文献复习,本文报告胎儿符合Ⅱ型OI。结论产前通过超声影像结合基因分型诊断胎儿为COL1A2基因新发突变（c.3142G〉T,***1048Cys）所致Ⅱ型OI;COL1A2基因编码蛋白长链双螺旋的400~480氨基酸及MLBR 3区域中甘氨酸被天冬氨酸或谷氨酸替代,多导致严重表型的OI;本文为产前准确预测胎儿结局、指导临床决策提供依据。