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Taking Cu-doped Ni2MnGa alloys as an example, the unified physical mechanism for martensitic phase transition and ductility in Ni–Mn-based ferromagnetic shape memory alloys was investigated theoretically.

Background Langerhans cell histiocytosis (LCH) is a rare neoplastic disease that occurs in both children and adults, and BRAF V600E is detected in up to 64% of the patients. Several studies have discussed the associations between BRAF V600E mutation and clinicopathological manifestations, but no clear conclusions have been drawn regarding the clinical significance of the mutation in pediatric patients. Results We retrieved the clinical information for 148 pediatric LCH patients and investigated the BRAF V600E mutation using next-generation sequencing alone or with droplet digital PCR. The overall positive rate of BRAF V600E was 60/148 (41%). The type of sample (peripheral blood and formalin-fixed paraffin-embedded tissue) used for testing was significantly associated with the BRAF V600E mutation status (p-value = 0.000 and 0.000). The risk of recurrence declined in patients who received targeted therapy (p-value = 0.006; hazard ratio 0.164, 95%CI: 0.046 to 0.583). However, no correlation was found between the BRAF V600E status and gender, age, stage, specific organ affected, TP53 mutation status, masses close to the lesion or recurrence. Conclusions This is the largest pediatric LCH study conducted with a Chinese population to date. BRAF V600E in LCH may occur less in East Asian populations than in other ethnic groups, regardless of age. Biopsy tissue is a more sensitive sample for BRAF mutation screening because not all of circulating DNA is tumoral. Approaches with low limit of detection or high sensitivity are recommended for mutation screening to avoid type I and II errors.

Mutations in the synaptic nuclear envelope protein 1 (SYNE1) gene have been reported to cause autosomal recessive cerebellar ataxia (ARCA) type 1 with highly variable clinical phenotypes. The aim of this study was to describe the phenotypic-genetic spectrum of SYNE1-related ARCA1 patients in the Chinese population. We screened 158 unrelated patients with autosomal recessive or sporadic ataxia for variants in SYNE1 using next-generation sequencing. Pathogenicity assessment of SYNE1 variants was interpreted according to the American College of Medical Genetics standards and guidelines. We identified eight truncating variants and two missense variants spreading throughout the SYNE1 gene from six unrelated families, including nine novel variants and one reported variant. Of the six index patients, two patients showed the classical pure cerebellar ataxia, while four patients exhibited non-cerebellar phenotypes, including motor neuron symptoms, cognitive impairment, or mental retardation. The variants associated with motor neuron or cognition involvement tend to be located in the C-terminal region of SYNE1 protein, compared with the variants related to pure cerebellar ataxia. Our data indicating SYNE1 mutation is one of the more common causes of recessive ataxia in the Chinese population. The use of next-generation sequencing has enabled the rapid analysis of recessive ataxia and further expanded our understanding of genotype-phenotype correlation.

Hereditary spastic paraplegias (HSP) are a group of rare neurodegenerative diseases characterized by progressive spastic paraparesis. UBAP1 was recently found to induce a rare type of HSP (SPG80). We identified a family with eight inherited spastic paraplegic patients carrying a novel heterozygous mutation c.279delG (p.S94Vfs*9) of UBAP1. We demonstrated a lack of functional UBAP1 in these patients, resulting in the neurological disorder caused by interceptions of the ESCRT pathway. Extending from the older onset-age identified from this family, we found that comparing with the European and other populations, Asian patients displayed less proportion of severe patients and an older average age at onset. The origins of SPG80 patients associated with both their onset age and their disease severity, while the age at onset was not correlated with the disease severity.

This study investigated the effects of microalgae growth on antibiotic removal and the attenuation of antibiotic resistance genes (ARGs)/ARGs host bacteria in algal-bacterial granular sludge (ABGS) system. In the presence of tetracycline (TC) and sulfadiazine (SDZ) mixture (2-4 mg/L), microalgae could grow on bacterial granular sludge (BGS) to form ABGS, with a chlorophyll-a content of 7.68-8.13 mg/g-VSS being achieved. The removal efficiencies of TC and SDZ by ABGS were as high as 79.0 % and 94.0 %, which were 4.3-5.0 % higher than those by BGS. Metagenomic analysis indicated that the relative abundances of TC/SDZ- related ARGs and mobile genetic elements (MGEs) in BGS were 56.1 % and 22.1 % higher than those in ABGS. A total of 26 ARGs were detected from the granules, and they were identified to associate with 46 host bacteria. 13 out of 26 ARGs and 13 out of 46 hosts were shared ARGs and hosts, respectively. The total relative abundance of host bacteria in BGS was 30.8 % higher than that in ABGS. Scenedesmus and Chlorella were the dominant microalgae that may reduce the diversity of ARGs hosts. Overall, ABGS is a promising biotechnology for antibiotic-containing wastewater treatment.

In this paper, we manufacture Ni50Ti25Al25 alloys dominated by L21-Ni2TiAl phases using mechanical alloying (MA) technique. The effects of process control agent (PCA) on MA process and mechanical properties of the Ni–Ti–Al alloys are investigated. It is found that the present L21-Ni50Ti25Al25 alloy exhibits high hardness, low reduced modulus and excellent pseudoelasticity. The PCA directly affects the MA process, microstructure and mechanical properties of the alloys. The MA sample without adding PCA has higher strength and much lower alloying temperature than that with PCA. Based on the nanoindentation tests, hardness of the both alloys showed obvious indentation depth dependence. Meanwhile a transition from normal indentation size effect of nano-hardness to reverse indentation size effect is observed.

SUMMARY: This study aimed to examine the differences in bone microarchitecture between different regions of the atlas in 28 dry atlas specimens using micro-CT, in order to explain the mechanism of the predilection sites of atlas fractures from the morphological point of view. A total of 28 dry specimens of intact adult atlas were randomly selected, scanned by micro-CT, and divided into a region from the anterior arch midpoint (AAM) to the lateral masses (LM), a LM region, and a region from the LM to the posterior arch midpoint (PAM). Trabecular thickness, separation, number, connectivity, and structure model index were measured for each of the three regions using the built-in software of the CT scanner. Trabecular thickness was all measured to be 0.11 ± 0.00 mm for AAM to LM, LM, and LM to PAM. Trabecular separation: AAM to LM > LM to PAM > LM. Trabecular number: LM > LM to PAM > AAM to LM. Connectivity: LM > LM to PAM > AAM to LM. Structure model index: LM > LM to PAM > AAM to LM. A lower trabecular number and connectivity and higher trabecular separation were seen in the anterior and posterior arches of the atlas, in which higher fracture rates were reported. By contrast, a higher trabecular number and connectivity and lower trabecular separation were seen in the lateral masses, in which lower fracture rates were reported.

Herein, we report a BiVO4 quantum dot (QDs)-decorated BiPO4 nanorods 0D/1D heterojunction via an in situ growth method. It showed enhanced visible-light-driven photocatalytic activity for degrading Rhodamine B (Rh B) compared to its pristine counterparts in composite. The mass ratio of BiVO4/BiPO4 was then varied from 3 wt% to 50 wt% and the optimum value was found to be 30 wt%, showing 8.3-fold and 6.3-fold apparent reaction rates higher than those of pristine BiPO4 and BiVO4, respectively. Moreover, all of the reduced Rh B was transformed into CO2 and H2O during the photocatalysis, thus showing the good mineralization ability (almost 100%) of the composite. Furthermore, the photocatalytic mechanism of the composite was also investigated here by the zeta potential, scavenger experiments, electron paramagnetic resonance (EPR), photoluminescence spectroscopy (PL), transient photocurrent and electrochemical impedance spectroscopy (EIS). Results show that (i) ˙OH was the main reactive species and (ii) BiVO4 could form a heterojunction with BiPO4, thus widening the response range to the visible region and accelerating the charge separation and transfer.

Mutations in the inositol 1,4,5-triphosphate receptor type 1 gene (ITPR1) lead to SCA15, SCA16, and SCA29. To date, only a few families with SCA29 have been reported. A three-generation Chinese family including four affected persons and two unaffected persons were enrolled in this study. We conducted whole-exome sequencing (WES) of the proband DNA initially to find the causal gene. We ascertained the family with autosomal dominant type of congenital nonprogressive cerebellar ataxia (CNPCA) associated with delayed motor and cognitive impairment. WES study was performed with two patients and identified c.1207-2A–T transition, in exon 14 of ITPR1, which was a splicing mutation. Sanger sequencing showed that four patients within this family carried the mutation and two unaffected members did not carry it. The results showed that the novel splicing mutation of ITPR1 was the causative gene for SCA29. In conclusion, we identified a novel SCA29 causative splicing mutation of ITPR1 in a Chinese family. We suggest ITPR1 gene analysis shall be a priority for diagnosis of patients with early-onset CNPCA. Our study demonstrated that whole-exome sequencing might rapidly improve the diagnosis of genetic ataxias. Electronic supplementary material The online version of this article (10.1007/s12311-017-0896-z) contains supplementary material, which is available to authorized users.

Single phase coarse-grained medium entropy alloys (MEA) generally possess a low yield strength and high plasticity at macroscopic scale, but strain hardening/softening behavior of nanocrystalline MEA still remains unknown. Here we design and prepare the nanolamellar CoCrNi MEA with heterogeneous interfaces by inserting a hard Ta thin layer. Interestingly, the CoCrNi/Ta pillars have an excellent combination with high yield strength and large uniform plastic strain as compared to single phase MEA when the layer thickness is below 50 nm. Moreover, the strength and strain hardening rate simultaneously increase with decreasing layer thickness in CoCrNi/Ta specimens. Strong constrain effect and the heterogeneous interface by the hard layer effectively suppress the propagation of major shear banding, which is responsible for the enhanced strain hardening.

NiTi thin films showed superplasticity with recovery strain up to 8%, however their strongly non-linear elasticity caused imprecise position control in MEMS requiring immediate and progressive control. Here, we report NiTi/W nanostructured multilayer films with tailorable stress window via layer spacing modulation. The stress window could be enlarged up to three times larger than that of single-layered NiTi film. Coherent phase boundary between B2 and α-W as well as the large transition strain of NiTi layer led to multilayers collaborative deformation without dislocation slip, making NiTi/W films large strain and strength. On the other hand, grain growth of NiTi layers was confined by W layers, thus layer spacing change could successfully modify the martensitic transition start stress of B2–B19’.

In this work, we have investigated the mechanical properties of Cu/Ta, Ag/Cu and Ag/Nb multilayers with different heterogeneous interfaces. The results suggest that when individual layer thickness (h) is larger than 5–10 nm, the hardness/strength of three different multilayer systems has the similar length scale effect with decreasing layer thickness, while when h ≤ 5 nm, the three multilayer systems show remarkably different plastic deformation behaviors. The strength curves exhibit the variation trends of unchanging, softening and increasing corresponding to Cu/Ta, Ag/Cu and Ag/Nb multilayers, respectively. The microstructure analysis shows that three kinds of multilayers have totally different interfacial structures, which lead to the different strengthening or softening mechanisms.

We summarized our minimally invasive thorascopic surgical experience of a rare congenital bronchobiliary fistula case and made a brief review of the literature. To our knowledge this is the first reported case treated by a total minimally invasive thorascopic surgery. Typical symptoms and signs, surgical experience, and pathological results were summarized with pictures. Intra-operative fistulography is of great importance to surgical decision making. Minimally invasive thorascopic surgery is safe and feasible in treating congenital bronchobiliary fistula without coexisting biliary tract malformation.

Herein, we report a BiVO4 quantum dot (QDs)-decorated BiPO4 nanorods 0D/1D heterojunction via an in situ growth method. It showed enhanced visible-light-driven photocatalytic activity for degrading Rhodamine B (Rh B) compared to its pristine counterparts in composite. The mass ratio of BiVO4/BiPO4 was then varied from 3 wt% to 50 wt% and the optimum value was found to be 30 wt%, showing 8.3-fold and 6.3-fold apparent reaction rates higher than those of pristine BiPO4 and BiVO4, respectively. Moreover, all of the reduced Rh B was transformed into CO2 and H2O during the photocatalysis, thus showing the good mineralization ability (almost 100%) of the composite. Furthermore, the photocatalytic mechanism of the composite was also investigated here by the zeta potential, scavenger experiments, electron paramagnetic resonance (EPR), photoluminescence spectroscopy (PL), transient photocurrent and electrochemical impedance spectroscopy (EIS). Results show that (i) ˙OH was the main reactive species and (ii) BiVO4 could form a heterojunction with BiPO4, thus widening the response range to the visible region and accelerating the charge separation and transfer.

The phase diagrams of continuous binary nanoalloys are important in providing guidance for material designs and industrial applications. However, experimental determination of the nano-phase diagram is scarce since calorimetric measurements remain quite challenging at the nanoscale. Based on the size-dependent cohesive energy model, we developed a unified nano-thermodynamic model to investigate the effects of the size, shape, and segregation on the phase diagrams of continuous binary nanoalloys. The liquidus/solidus dropped in temperature, two-phase zone was narrowed, and the degree of surface segregation decreased with decrease in the size or increase in the shape factor. The congruent melting point of Cu-Au nanoalloys with and without segregation is linearly shifted to higher Au component and lower temperature with decreasing size or increasing shape factor. By reviewing surface segregated element of different binary nanoalloys, two segregation rules based on the solid surface energy and atomic size have been identified. Moreover, the established model can be employed to describe other physicochemical properties of nanoalloys, e.g. the cohesive energy, catalytic activation energy, and order-disorder transition temperature, and the validity is supported by available other theoretical prediction, experimental data and molecular dynamic simulations results. This will help the experimentalists by guiding them in their attempts to design bimetallic nanocrystals with the desired properties.

The size dependence of phase transformation behaviors were investigated for nanoscale TiNi thin films. It was found for the first time that size reduction favored B2–R phase transformation and that R-phase starting temperature (Rs) exhibited abnormal change with film thickness and grain size decreasing. The influence of grain refinement and Ni-enrichment zone was dominant for thickness between 300 and 90 nm, and substrate-induced internal stress played crucial role as thickness were below 90 nm, resulting in the initial decrease and final increase of Rs.

Nanoindentation was conducted for magnetron sputtered TiNi thin film, which exhibits different mechanical responses induced by varied indenter shapes. Superelasticity and incomplete phase transition (A → R) were clearly depicted by spherical indentation. Under the peak load of 500 μN, the depth recovery ratios are 100% and 68% determined by spherical and Berkovich indentations, respectively. The different response is due to the varied stress distribution beneath two types of indenters. Large plastic deformation hinders the reverse phase transition (R → A) in Berkovich indentation, resulting lower recovery. Moreover, significant difference in indentation size effect for TiNi film was revealed comparing to traditional metals. And phase transition contributed considerably to the abnormal hardness evolution.

Nickel and silver particles were prepared by using sol–gel auto-combustion method under N2 atmosphere where lactic acid was applied as chelating agent. The synthesis of nickel particles was carried out at various pH conditions (2–7), resulting in the face-centered-cubic or hexagonal-close-packed crystalline nickel phase. The morphology and structure of synthesized nickel particles and silver nanoparticles were characterized by X-ray diffraction, transmission electron microscope, energy dispersive X-Ray spectroscopy and differential scanning calorimetry-thermogravimetric analysis. The results show that the spherical Ag nanoparticles with diameters in the range of 18–27 nm and narrow size distribution can be obtained by this sol–gel process.

Cyclic deformation behavior of submicron-thick austenitic TiNi film at nanoscale was studied by nanoindentation. It was found that excellent superelasticity was achieved and stabilized after certain times of indentation deformation, and lower loading would require more deformation cycles to achieve superelasticity stabilization. Loss of superelasticity was attributed to the lower yield strength of austenite than the critical stress required for stress-induced martensitic transformation. Both introduced dislocations and grain growth during cyclic deformations contributed to the superelasticity and its steady-state achievement.

BACKGROUND No/slow reflow gives rise to serious complications in STEMI patients undergoing PCI, and can lead to worse outcomes. Several measures are used to prevent no/slow reflow, including thrombus removal processes and intensive use of anticoagulant agents. Our study was designed to evaluate the efficacy and safety of thrombectomy and intracoronary administration of GPIIb/IIIa inhibitors in STEMI patients undergoing PPCI. MATERIAL AND METHODS We randomly assigned 240 STEMI patients into 3 groups. Before PPCI, patients in group A received thrombectomy and intracoronary administration of tirofiban. Patients in group B received thrombectomy, and patients in group C neither of these 2 treatments. Their demographic data and coronary angiography results were recorded. TIMI grade flow was used to evaluate the effect. After the follow-up, major adverse cardiac events were regarded as study endpoints in evaluating the safety of the combined therapy. RESULTS We found no significant differences among the 3 groups in demographic and clinical characteristics (p>0.05). Patients in group A had better TIMI grade classifications and ST-segment elevation (p=0.005), and lower incidence of no/slow reflow (p=0.031) and MACE. During 6-month follow-up, the MACE rate was lower in group A than in groups B and C (p=0.038). CONCLUSIONS The use of thrombectomy combined with intracoronary administration of tirofiban is relatively effective and safe in STEMI patients undergoing PPCI.

Three-dimensional (3D) flowerlike silver microparticles (FSMPs) with novel nanostructure have been synthesized on the surface of a Pt film by a very simple, rapid and cost-effective electrochemical approach without introducing any template, surfactant or modification of electrode surfaces. The as-prepared FSMPs exhibit flowerlike morphologies and are built with many 2D nanoflakes as building blocks. The nanoflakes intersect mutually and have smooth surfaces, outwardly wavy edges and uniform thickness. The density of nanoflakes and the diameter of the FSMPs can be easily controlled by changing the electrodeposition time. A possible mechanism for electrochemical growth of the flake-built FSMPs is suggested on the basis of experimental results.

Plants harbor complex and variable microbial communities. Using molecular-based techniques targeting the 16S rRNA gene, we studied the developmental stages and geographical location diversity of endophytic archaea in two locations (Shihezi and Changji) and four periods (the seedling growth, rosette formation, tuber growth and sucrose accumulation sampling periods) in the north slope of Tianshan Mountain, China. Community structure of mixed sample from 60 sugar beet plants was examined using PCR-based 454 pyrosequencing and terminal restriction fragment length polymorphism (T-RFLP). In total, 5290 archaea 16S rRNA sequences were obtained from all sugar beet samples. The most abundant archaea groups in all sugar beet were Methanococci, the miscellaneous Crenarchaeotic Group and Thermoplasmata. There was a marked difference in diversity of endophytic archaea in sugar beet for different growth periods. The greatest number of Operational T-RFLP Units (OTUs) was detected during sucrose accumulation (298) and rosette formation (282). Endophytic archaea diversity was reduced during seedling growth (128 OTUs) and tuber growth (55 OTUs). Nine OTUs were common to all four periods of growth. There were more OTUs in Shihezi than in Changji. Clustering analysis and principal component analysis of T-RFLP data revealed distinct shifts in endophytic archaea community profiles that corresponded to plant growth stage rather than geographical location. The dynamics of endophytic archaea communities were influenced by plant growth stage. To our knowledge, this is the first report that archaea has been identified as endophytes associated with sugar beet by the culture-independent approach. The results suggest that the diversity of endophytic archaea is abundant in sugar beet.

Transcription elongation has been recognized as a rate-limiting step for the expression of signal-inducible genes. Through recruitment of positive transcription elongation factor P-TEFb, the bromodomain-containing protein BRD4 plays critical roles in regulating the transcription elongation of a vast array of inducible genes that are important for multiple cellular processes. The diverse biological roles of BRD4 have been proposed to rely on its functional transition between chromatin targeting and transcription regulation. The signaling pathways and the molecular mechanism for regulating this transition process, however, are largely unknown. Here, we report a novel role of phosphorylated Ser(10) of histone H3 (H3S10ph) in governing the functional transition of BRD4. We identified that the acetylated lysines 5 and 8 of nucleosomal histone H4 (H4K5ac/K8ac) is the BRD4 binding site, and the protein phosphatase PP1α and class I histone deacetylase (HDAC1/2/3) signaling pathways are essential for the stress-induced BRD4 release from chromatin. In the unstressed state, phosphorylated H3S10 prevents the deacetylation of nucleosomal H4K5ac/K8ac by HDAC1/2/3, thereby locking up the majority of BRD4 onto chromatin. Upon stress, PP1α-mediated dephosphorylation of H3S10ph allows the deacetylation of nucleosomal H4K5ac/K8ac by HDAC1/2/3, thereby leading to the release of chromatin-bound BRD4 for subsequent recruitment of P-TEFb to enhance the expression of inducible genes. Therefore, our study revealed a novel mechanism that the histone cross-talk between H3S10ph and H4K5ac/K8ac connects PP1α and HDACs to govern the functional transition of BRD4. Combined with previous studies on the regulation of P-TEFb activation, the intricate signaling network for the tight control of transcription elongation is established.