A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2

We report heterozygous mutations in the genes encoding either type I or type II transforming growth factor beta receptor in ten families with a newly described human phenotype that includes widespread perturbations in cardiovascular, craniofacial, neurocognitive and skeletal development. Despite evidence that receptors derived from selected mutated alleles cannot support TGFbeta signal propagation, cells derived from individuals heterozygous with respect to these mutations did not show altered kinetics of the acute phase response to administered ligand. Furthermore, tissues derived from affected individuals showed increased expression of both collagen and connective tissue growth factor, as well as nuclear enrichment of phosphorylated Smad2, indicative of increased TGFbeta signaling. These data definitively implicate perturbation of TGFbeta signaling in many common human phenotypes, including craniosynostosis, cleft palate, arterial aneurysms, congenital heart disease and mental retardation, and suggest that comprehensive mechanistic insight will require consideration of both primary and compensatory events.     

New weighting factors assignment of evidence theorybased one vidence distance

1.INTRODUCTION Dempster Shafertheoryiswidelyusedinmanyfields suchasinformationfusionanddecision making[1,2].Anditisaverysimpleandstraightforwardwayofex tendingclassicalprobabilitytheory.However,Demp ster’scombinationoperatorisapoorsolutionforthe managementoftheconflictbetweenthevariousinfor mationsourcesatthenormalizationstep.Severalau thorshavecriticizeditandsomehaveproposedother solutions[3,4].InRef.[4],Lefevrehaspointedout thatthecoreofalotofmodifiedmethodistheassign mentoftheconfli…     

Urban transport policy response to global climate change in China

Accompanying with the economic development and the process of industrialization and urbanization in China, the energy consumption and greenhouse gases emission of transport sector increased rapidly. From the viewpoint of how the urban traffic management department should cope with the global climate change, based on the economy, institutional reform, planning and policies in China, the paper analyzed the main important problems and obstacles in the development of urban transport, put forward the corresponding policy recommendations which could lead the urban transport system transform to low carbon emission and become more suitable to the climate change.     

Computational diffusion kinetics and its applications in study and design of rare metallic materials

Computational diffusion kinetics(CDK),with a spirit of and being coupled with the computational thermodynamics(CT,or called as the CALPHAD technique),plays increasingly important role in the alloy design/optimization and microstructure control during the processing of advanced metallic materials.This paper is to highlight recent progress of CDK in research with great focus on novel Ti and Zr alloys,which was largely performed in the authors’group.It ends with one representative example of the applications of CDK,coupled with CT,quantitative phase field,and three-dimensional(3D)statistical calculation,in designing the heattreatment schedule for the dual phase(αβ)Ti–6Al–4V alloys.     

Photocatalytic enhancement of Mg-doped ZnO nanocrystals hybridized with reduced graphene oxide sheets

Hybridization of Mg-doped ZnO and reduced graphene oxide(MZO–RGO) were synthesized through one pot reaction process. Crystallization of MZO–RGO upon thermal decomposition of the stearate precursors was investigated by X-ray diffraction technique. XRD studies point toward the particles size with 10–15 nm,which was confirmed by transmittance electronic microscopy,and also indicates that graphene oxide has been directly reduced into its reduced state graphene during the synthesis. Graphene hybridized MZO photocatalyst showed enhanced catalytic activity for the degradation of methylene blue(MB). The degree of photocatalytic activity enhancement strongly depended both on the coverage of graphene on the surface of MZO nanoparticles and the Mg doping concentration. The sample of 2 wt% graphene hybridized 5 at% Mg-doped ZnO showed the highest photocatalytic activity,which remained good photocatalytic activity after nine cycling runs.     

Joining of Si3N4 ceramic using PdCo(Ni SiB)–V system brazingfi ller alloy and interfacial reactions

The wettability of V-active PdCo-based alloys on Si3N4ceramic was studied with the sessile drop method. And the alloy of Pd50.0–Co33.7–Ni4.0–Si2.0–B0.7–V9.6(wt%),was developed for Si3N4ceramic joining in the present investigation. The rapidly-solidified brazing foils were fabricated by the alloy Pd50.0–Co33.7–Ni4.0–Si2.0–B0.7–V9.6. The average room-temperature three-point bend strength of the Si3N4/Si3N4joints brazed at 1453 K for 10 min was 205.6 MPa,and the newly developed braze gives joint strengths of 210.9 MPa,206.6 MPa and 80.2 MPa at high temperatures of 973 K,1073 K and 1173 K respectively. The interfacial reaction products in the Si3N4/Si3N4joint brazed at 1453 K for10 min were identified to be VN and Pd2Si by XRD analysis. Based on the XEDS analysis result,the residual brazing alloy existing at the central part of the joint was verified as Co-rich phases,in which the concentration of element Pd was high up to 18.0–19.1 at%. The mechanism of the interfacial reactions was discussed. Pd should be a good choice as useful alloying element in newer high-temperature braze candidates for the joining of Si-based ceramics.     

All-inorganic quantum-dot light-emitting-diodes with vertical nickel oxide nanosheets as hole transport layer

All-inorganic quantum dot light emitting diodes (QLEDs) have gained great attention as a result of their high stability under oxygen-rich, humid and high current working conditions. In this work, we have fabricated an all-inorganic QLED device (FTO/NiO/QDs/AZO/Ag) with sandwich-structure, wherein the inorganic metal oxides thin films of NiO and AZO were employed as hole and electron transport layers, respectively. The porous NiO layer with vertical lamellar nanosheets interconnected microstructure have been directly synthesized on the substrate of conductive FTO glass and increased the wettability of CdSe@ZnS QDs, which result in an enhancement of current transport performance of the QLED.     

A NEW MERMITHID PARASITE OF THE BLACK CUTWORM

A new species of mermithid nematodes was collected from theinfected larvae of Agrotis ipsilon in Anqing,Anhui in 1984-1985.It is identif-ied as new species of the genus Hexamermis.Hexamermis agrotis sp.nov.     

Enhanced recovery of high-purity Fe powder from iron-rich electrolytic manganese residue by slurry electrolysis

Iron-rich electrolytic manganese residue (IREMR) is an industrial waste produced during the processing of electrolytic metal manganese,and it contains certain amounts of Fe and Mn resources and other heavy metals.In this study,the slurry electrolysis technique was used to recover high-purity Fe powder from IREMR.The effects of IREMR and H₂SO₄ mass ratio,current density,reaction temperature,and electrolytic time on the leaching and current efficiencies of Fe were studied.Acc...     

Simple construction of the α-MnO2@ZIF-8 nanomaterial with highly enhanced U(Ⅵ) adsorption performance and assessed removal mechanism

In this study, the one-dimensional nanomaterial α-MnO₂@ZIF-8 was satisfactorily fabricated using a simple hydrothermal method by modifying ZIF-8 on α-MnO₂ nanowires for high-performance extraction of U(Ⅵ) out of water solution. The experimental data indicated that the adsorption of U(Ⅵ) on α-MnO₂@ZIF-8 was intensely dependent on solution pH but not on ionic strength. The maximum adsorption of U(Ⅵ) on α-MnO₂@ZIF-8 at pH= 5.0 and T = 298 K was 802.36 mg/...