A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, ‘chicken’skin appearance and perforating elastosis serpiginosalike lesions in the genitocrural regi...A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, ‘chicken’skin appearance and perforating elastosis serpiginosalike lesions in the genitocrural region. Histologically, calcified material perforating the epidermis and adjacent short curled and mineralized elastic fibres suggested a variant of pseudoxanthoma elasticum (PXE). As PXE is known to be caused by various mutations in the transmembrane transporter ABCC6 gene, we hypothesized that a novel ABCC6 mutation may underlie this unique combination of PXE and elastopathic vascular damage. Therefore, the complete ABCC6 coding region of the patient and her parents was screened for genetic alterations. No bona fide disease-causing mutation of ABCC6 could be found in the child and in her parents. However, two novel allelic amino acid substitutions (Arg1273Lys and Glu1293Lys; exon 27) were found in the girl and her father, localized in close proximity to the region that codes for the functionally critical second nucleotide-binding fold of ABCC6. Although a causal involvement of these amino acid substitutions could not be proven based on this study, both heterozygote substitutions may possibly have interacted with other undetected recessive maternal ABCC6 changes in the child. To the best of our knowledge, this is the first report of an association between early-onset PXE and severe Moya Moya syndrome possibly related to ABCC6 changes.展开更多
With titanium-bearing blast furnace slag, ammonium sulfate, citric acid and potassium carbonate as raw materials to produce foliar fertilizer and Ca-S-Si compound fertilizer by means of heating process for the first t...With titanium-bearing blast furnace slag, ammonium sulfate, citric acid and potassium carbonate as raw materials to produce foliar fertilizer and Ca-S-Si compound fertilizer by means of heating process for the first time, which improved the solubility of the slag and converted the nutritional elements into such ones which are prone to be absorbed by plants. The effects of process conditions on dissolution rate of titanium were mainly analyzed through the orthogonal experiment. The results showed that the optimum synthesis process conditions of foliar fertilizer from the slag were 360 ℃ for 35 min, the mass ratio of ammonium sulfate and titanium-bearing blast furnace slag was 8：1. Under these conditions more than 80% of iron, titanium, magnesium and part of silicon in titanium-bearing blast furnace slag converted into water-soluble substances and existing in foliar fertilizer. Foliar fertilizer contained nitrogen, sulfur, potassium, iron, titanium, magnesium and silicon, and pH value of foliar fertilizer was 6. Ca-S-Si compound fertilizer mainly contained calcium silicate and calcium sulfate, which existed in the form of citric acid-soluble substance and slightly soluble substance, respectively.展开更多
Despite the intense research efforts directed to electrocatalytic nitrogen reduction reaction(eNRR),the NH_(3) yield and selectivity are still not up to the standard of practical application.Here,high-entropy perovski...Despite the intense research efforts directed to electrocatalytic nitrogen reduction reaction(eNRR),the NH_(3) yield and selectivity are still not up to the standard of practical application.Here,high-entropy perovskite oxides with composition Bax(FeCoNiZrY)_(0.2)O_(3−δ)(Bx(FCNZY)_(0.2)(x=0.9,1)are reported as eNRR catalysts.The eNRR activity of high-entropy perovskite oxide is enhanced by changing the nonstoichiometric metal elements at the A-site,thus generating additional oxygen vacancies.The NH_(3) yield and Faraday efficiency for B_(0.9)(FCNZY)_(0.2) are 1.51 and 1.95 times higher than those for B(FCNZY)_(0.2),respectively.The d-band center theory is used to theoretically predict the catalytically active center at the B-site,and as a result,nickel was identified as the catalytic site.The free energy values of the intermediate states in the optimal distal pathway show that the third protonation step(*NNH_(2)→*NNH_(3))is the rate-determining step and that the increase in oxygen vacancies in the high-entropy perovskite contributes to nitrogen adsorption and reduction.This work provides a framework for applying high-entropy structures with active site diversity for electrocatalytic nitrogen fixation.展开更多
Epitaxial heterostructures based on organicinorganic hybrid perovskites and two-dimensional materials hold great promises in optoelectronics, but they have been prepared only via solid-state methods that restricted th...Epitaxial heterostructures based on organicinorganic hybrid perovskites and two-dimensional materials hold great promises in optoelectronics, but they have been prepared only via solid-state methods that restricted their practical applications. Herein, we report cubic-phased MAPbBr3(MA=CH3NH3+) nanocrystals were epitaxially deposited on trigonal/hexagonal-phased MoS2 nanosheets in solution by facilely tuning the solvation conditions. In spite of the mismatched lattice symmetry between the square MAPbBr3(001) overlayer and the hexagonal MoS2(001) substrate, two different aligning directions with lattice mismatch of as small as 1% were observed based on the domainmatching epitaxy. This was realized most likely due to the flexible nature and absence of surface dangling bonds of MoS2 nanosheets. The formation of the epitaxial interface affords an effective energy transfer from MAPbBr3 to MoS2, and as a result, paper-based photodetectors facilely fabricated from these solution-dispersible heterostructures showed better performance compared to those based on MoS2 or MAPbBr3 alone. In addition to the improved energy transfer and light adsorption, the use of MoS2 nanosheets provided flexible and continuous substrates to connect the otherwise discrete MAPbBr3 nanocrystals and achieved the better film forming ability. Our work suggests that the scalable preparation of heterostructures based on organic-inorganic hybrid perovskites and 2D materials via solution-phase epitaxy may bring about more opportunities for expanding their optoelectronic applications.展开更多
As the technological development of large single-crystalline wafers have revolutionized many industries including electronics and photovoltaics,one can predict that the availability of large single-crystalline perovsk...As the technological development of large single-crystalline wafers have revolutionized many industries including electronics and photovoltaics,one can predict that the availability of large single-crystalline perovskite crystals and wafers can revolutionize its broad applications in photodetectors,solar cells,LEDs,lasers,etc.In 2015,Liu et al.展开更多
文摘A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, ‘chicken’skin appearance and perforating elastosis serpiginosalike lesions in the genitocrural region. Histologically, calcified material perforating the epidermis and adjacent short curled and mineralized elastic fibres suggested a variant of pseudoxanthoma elasticum (PXE). As PXE is known to be caused by various mutations in the transmembrane transporter ABCC6 gene, we hypothesized that a novel ABCC6 mutation may underlie this unique combination of PXE and elastopathic vascular damage. Therefore, the complete ABCC6 coding region of the patient and her parents was screened for genetic alterations. No bona fide disease-causing mutation of ABCC6 could be found in the child and in her parents. However, two novel allelic amino acid substitutions (Arg1273Lys and Glu1293Lys; exon 27) were found in the girl and her father, localized in close proximity to the region that codes for the functionally critical second nucleotide-binding fold of ABCC6. Although a causal involvement of these amino acid substitutions could not be proven based on this study, both heterozygote substitutions may possibly have interacted with other undetected recessive maternal ABCC6 changes in the child. To the best of our knowledge, this is the first report of an association between early-onset PXE and severe Moya Moya syndrome possibly related to ABCC6 changes.
基金This study was supported by the National Natural Science Foundation of China （No. 50874029） and the National Basic Research Program of China （No.2007CB613504）. Thanks are due to X.L. Nan and B.Y. Ma for their assistance in language editing.
文摘With titanium-bearing blast furnace slag, ammonium sulfate, citric acid and potassium carbonate as raw materials to produce foliar fertilizer and Ca-S-Si compound fertilizer by means of heating process for the first time, which improved the solubility of the slag and converted the nutritional elements into such ones which are prone to be absorbed by plants. The effects of process conditions on dissolution rate of titanium were mainly analyzed through the orthogonal experiment. The results showed that the optimum synthesis process conditions of foliar fertilizer from the slag were 360 ℃ for 35 min, the mass ratio of ammonium sulfate and titanium-bearing blast furnace slag was 8：1. Under these conditions more than 80% of iron, titanium, magnesium and part of silicon in titanium-bearing blast furnace slag converted into water-soluble substances and existing in foliar fertilizer. Foliar fertilizer contained nitrogen, sulfur, potassium, iron, titanium, magnesium and silicon, and pH value of foliar fertilizer was 6. Ca-S-Si compound fertilizer mainly contained calcium silicate and calcium sulfate, which existed in the form of citric acid-soluble substance and slightly soluble substance, respectively.
基金supported by the National Natural Science Foundation of China (52161135302, 21674019, and 51801075)the Research Foundation Flanders (G0F2322N)+8 种基金Shanghai Scientific and Technological Innovation Project (18JC1410600)the Program of the Shanghai Academic Research Leader (17XD1400100)the financial support from the Flemish Government through the Moonshot cSBO project P2C (HBC.2019.0108)the Long-term Structural Funding (Methusalem CASAS2, Meth/15/04)Interne Fondsen KU Leuven through project C3/20/067the support from the Research Foundation-Flanders (FWO) in the form of a doctoral fellowship (1SA3321N)the financial support from China Scholarship Council in the form of a visiting Ph.D. Student (File No. 202106790090)the LvLiang Cloud Computing Center of China, and the calculations were performed on a TianHe-2 systemthe characterizations supported by the Central Laboratory, School of Chemical and Material Engineering, Jiangnan University。
文摘Despite the intense research efforts directed to electrocatalytic nitrogen reduction reaction(eNRR),the NH_(3) yield and selectivity are still not up to the standard of practical application.Here,high-entropy perovskite oxides with composition Bax(FeCoNiZrY)_(0.2)O_(3−δ)(Bx(FCNZY)_(0.2)(x=0.9,1)are reported as eNRR catalysts.The eNRR activity of high-entropy perovskite oxide is enhanced by changing the nonstoichiometric metal elements at the A-site,thus generating additional oxygen vacancies.The NH_(3) yield and Faraday efficiency for B_(0.9)(FCNZY)_(0.2) are 1.51 and 1.95 times higher than those for B(FCNZY)_(0.2),respectively.The d-band center theory is used to theoretically predict the catalytically active center at the B-site,and as a result,nickel was identified as the catalytic site.The free energy values of the intermediate states in the optimal distal pathway show that the third protonation step(*NNH_(2)→*NNH_(3))is the rate-determining step and that the increase in oxygen vacancies in the high-entropy perovskite contributes to nitrogen adsorption and reduction.This work provides a framework for applying high-entropy structures with active site diversity for electrocatalytic nitrogen fixation.
基金supported by the National Natural Science Foundation of China (51322202) the Young 1000 Talents Global Recruitment Program of China+2 种基金the financial support from Macao Science and Technology Development Fund (FDCT-116/2016/A3 and FDCT-091/2017/A2)Research Grant (SRG2016-00087-FST) from the University of Macao, the Natural Science Foundation of China (91733302, 61605073 and 2015CB932200)the Young 1000 Talents Global Recruitment Program of China
文摘Epitaxial heterostructures based on organicinorganic hybrid perovskites and two-dimensional materials hold great promises in optoelectronics, but they have been prepared only via solid-state methods that restricted their practical applications. Herein, we report cubic-phased MAPbBr3(MA=CH3NH3+) nanocrystals were epitaxially deposited on trigonal/hexagonal-phased MoS2 nanosheets in solution by facilely tuning the solvation conditions. In spite of the mismatched lattice symmetry between the square MAPbBr3(001) overlayer and the hexagonal MoS2(001) substrate, two different aligning directions with lattice mismatch of as small as 1% were observed based on the domainmatching epitaxy. This was realized most likely due to the flexible nature and absence of surface dangling bonds of MoS2 nanosheets. The formation of the epitaxial interface affords an effective energy transfer from MAPbBr3 to MoS2, and as a result, paper-based photodetectors facilely fabricated from these solution-dispersible heterostructures showed better performance compared to those based on MoS2 or MAPbBr3 alone. In addition to the improved energy transfer and light adsorption, the use of MoS2 nanosheets provided flexible and continuous substrates to connect the otherwise discrete MAPbBr3 nanocrystals and achieved the better film forming ability. Our work suggests that the scalable preparation of heterostructures based on organic-inorganic hybrid perovskites and 2D materials via solution-phase epitaxy may bring about more opportunities for expanding their optoelectronic applications.
文摘As the technological development of large single-crystalline wafers have revolutionized many industries including electronics and photovoltaics,one can predict that the availability of large single-crystalline perovskite crystals and wafers can revolutionize its broad applications in photodetectors,solar cells,LEDs,lasers,etc.In 2015,Liu et al.
