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As a highly conserved serine/threonine kinase with catalytic and regulatory subunits distributed ubiquitously in eukaryotic organisms, casein kinase 2 (CK2) is involved in multiple cellular functions, including immune regulation. In this study, two variants of the catalytic subunit (designated PvCK2α-1 and PvCK2α-2) and the regulatory subunit homologs (designated PvCK2β-1 and PvCK2β-2) in Penaeus vannamei were cloned and characterised. PvCK2α-1 and PvCK2α-2 shared the same genomic sequence consisting of six exons and five introns and encoded the same protein of 350 amino acids with an S_TKc domain, although there was a sequence deletion in 3'-UTR in PvCK2α-2 when compared with PvCK2α-1. Because of the sequence deletion in the ORF, PvCK2β-1 and PvCK2β-2 encoded different proteins with a CK_II_beta domain. The gene structures of PvCK2β-1 and PvCK2β-2 were identical and consisted of four exons and three introns. Semi-quantitative RT-PCR analyses revealed that PvCK2α and PvCK2β were constitutively expressed in all P. vannamei tissues tested, with higher levels detected in the immune-related tissues including hemocytes, hepatopancreas, gills and intestine. In these four tissue types, all variants of PvCK2α and PvCK2β were induced upon challenge with white spot syndrome virus (WSSV), Vibrio parahaemolyticus and Staphyloccocus aureus. The inhibition of PvCK2α, PvCK2β-1 and PvCK2βComb (the amount of PvCK2β-1 and PvCK2β-2) significantly reduced the survival rates of P. vannamei after WSSV infection and significantly increased the WSSV viral loads. Knockdown of PvCK2 by RNAi could distinctly decrease the expression of NF-κB related genes. All of these results suggest that PvCK2 plays an important role in the innate immune response to pathogen challenges in P. vannamei, with a positive role in anti-WSSV response which may be mediated through regulating the expression of NF-κB drived antimicrobial peptide genes.

High smelting temperature of phosphate ore and low activity of the slag lead to energy-extensive consumption and large solid waste generation in the yellow phosphorus industry. Micronutrient-bearing cosolvents were added to the reduction smelting process of phosphate ore to reduce the smelting temperature and increase the activity of slag in the current work. Thermodynamic analysis demonstrated that B2O3 and K2O as cosolvents can decrease the melting temperature, promote the liquid phase formation and reduce the slag viscosity. In addition, cosolvents had no unfavorable effect on the reduction volatilization of phosphate ore, and the volatilization ratio of phosphorus was > 97 wt.% at 1550°C for 1 h. In this case, the value of slag was enhanced with the addition of cosolvents, and the activation ratios of micronutrients were > 90%. Resultantly, no solid waste was generated since the activated slag can be regarded as a favorable ingredient for the crop fertilizer.

Patient reported outcomes in Implantable Cardioverter Defibrillator (ICD) patients can describe the experience of living with heart disease and with an ICD. However, very little is known about patient outcomes among Chinese patients which may limit effective patient discussions and interventions for these patients. The purposes of this study were to examine device related experiences (e.g., device acceptance, shock anxiety) in Chinese ICD patients and identify potential variables that influence health related quality of life (HRQOL) and to compare HRQOL outcomes to healthy and heart failure populations.This study used a cross-sectional research design with serially recruited ICD patients (N = 100) from clinics in China. Participants completed surveys including: the 12-Item Short-Form Health Survey Questionnaire (SF-12), Florida Patient Acceptance Survey (FPAS), Florida Shock Anxiety Scale (FSAS), Type D Scale (DS-14), and general information questionnaire.Participants were 100 ICD patients in China with a mean age of 53.32(SD = 13.70). The mean scores of the SF-12 physical component summary (PCS) and mental component summary (MCS)of ICD patients (43.55 and 47.07, respectively) were lower than the Chinese general population (P0.001) and general health, social functioning, and role emotional scores were lower than chronic heart failure patients (P0.001). Multiple linear regression analysis indicated that LVEF, positive shock history, age and shock anxiety were significant predictors of physical function and accounted for 24.5% of the adjusted variance. Type D personality, shock history, and shock anxiety were predictors of the mental health component and accounted for 25.9% of the variance. Shock history, age, type D personality, and shock anxiety significantly predicted device acceptance (FPAS-Total) and accounted for 32% of variance.ICD patients reported health outcomes were generally lower than the Chinese general population and patients with heart failure in relation to general health, social functioning, and role emotional. Both generic and disease specific HRQOL were influenced by both medical and psychosocial predictors. This suggests that current Western society based comprehensive models of patient HRQOL and patient care needs may extend to Chinese patients with ICDs.

Pacing the cardiac conduction system has been explored in patients with conduction system disease, but comprehensive comparisons between different pacing modalities are not well investigated. To compare pacing characteristics and ventricular synchrony between His-bundle pacing (HBP) and left bundle branch pacing (LBBP) in patients with atrioventricular block (AVB). Fifty pacemaker-indicated patients with AVB were enrolled. Twenty-five patients underwent HBP, and another 25 patients underwent LBBP. Success rate, procedural and fluoroscopy duration, pacing parameters, and echocardiographic data were perioperatively assessed and at 3-month follow-up. HBP was successful in 19 of 25 (76.0%) patients, whereas LBBP was successful in 22 of 25 (88.0%) patients. Compared with HBP, LBBP capture threshold was significantly lower (0.76 ± 0.25 V/0.4 ms vs. 1.27 ± 0.61 V/1.0 ms, P = 0.003) and R-wave amplitude was significantly higher with LBBP (11.7 ± 6.6 vs. 4.9 ± 2.4 mV, P

Biochemical fulvic acid (BFA), produced by organic wastes composting, is the complex organic matter with various functional groups. A novel modified biochemical fulvic acid (MBFA) which possessed stronger chelating ability had been synthesized by the grafting copolymerization of BFA and acrylic acid (AA). Results showed that MBFA effectively inhibited the crystallization of calcium phosphate and increased the concentration of phosphate in water solution. The optimum reaction conditions optimized by Box–Behnken design and response surface methodology were reaction temperature 69.24 °C, the mass of monomer to fulvic acid ratio 0.713, the initiator dosage 19.78%, and phosphate crystal-inhibition extent was 96.89%. IR spectra demonstrated AA was grafted onto BFA. XRD data and SEM images appeared the formation and growth of calcium phosphate crystals was effectively inhibited by MBFA.

In this paper, a novel process is proposed for extracting rare earth elements through intensifying the decomposition of mixed rare earth concentrates by microwave heating. The objective of the present study was to investigate the phase transition mechanism of a mixed rare earth concentrate with sodium hydroxide in a microwave field. To this end, the decomposition and separation efficiencies of the rare earth elements and fluorine were analyzed. The results showed that the decomposition reaction of the tested mixed rare earth concentrate in the microwave irradiation field occurred in three stages. The rare earth elements initially presented in bastnaesite and monazite were gradually converted to rare earth hydroxides (RE(OH)3), rare earth oxides (RE2O3), and composite oxides (Ce0.5Nd0.5O1.75), with fluorine eventually forming sodium fluoride. The decomposition ratio of the mixed concentrate and conversion ratio of the fluoride reached 88.73% and 89.32%, respectively. Furthermore, the decomposition temperature of the mixed concentrate with microwave heating was lower than that required with conventional heating, which indicated that microwave heating could decrease the thermodynamic reaction temperature. The results of the microscopic morphology analysis showed that after microwave heating, the samples exhibited two distinct particle morphologies, which was different from that of a mixed concentrate in any case. The decompositions of bastnaesite, monazite, and fluorite were somewhat non-interfering in nature, and sodium fluoride and calcium phosphate were separated from the rare earth oxides in the decomposition products. These results indicated that the proposed process was conducive to the separation of rare earth elements and fluoride and helpful for improving the efficiency of rare earth leaching.

Antimicrobial peptides (AMPs) play an important role in the host defense system of shrimps. In this study, an Arasin-like peptide, named as LvArasin-like, was identified from the hemocytes of the pacific white shrimp, Litopenaeus vannamei. The complete open reading frame (ORF) of LvArasin-like was 213 bp, encoding 70 amino acid residues with a predicted molecular mass of 5.68 kDa and a theoretical isoelectric point (pI) of 6.73. The predicted peptide consisted of a signal peptide, an N-terminal Pro/Arg-rich domain, and a C-terminal cysteine-rich domain. LvArasin-like expression was most abundant in the gills and was up-regulated in hemocytes after LPS or Poly I:C injection as well as challenges by Vibrio parahaemolyticus or Staphylococcus aureus infection. In the heterologous expression system, LvArasin-like protein (rLvArasin-like) was recombinantly expressed in the forms of a dimer or both a monomer and dimer. The rLvArasin-like could directly bind to gram-positive and gram-negative bacteria and exhibited broad-spectrum antimicrobial activity towards them, with 50 % of minimal inhibitory concentrations (MIC50) of 6.25–50 μM. Moreover, dsRNA-mediated knockdown of LvArasin-like enhanced the susceptibility of shrimp to V. parahaemolyticus. In addition, the transcriptional level of LvArasin-like was downregulated when silencing of the transcription factors LvDorsal and LvRelish using RNAi in vivo. All of these results suggest that LvArasin-like is involved in host defense against bacterial infection. Therefore, it is a potential therapeutic agent for disease control in shrimp aquaculture.

Yellow phosphorus produced by electric furnace is an important raw material for the production of high-purity phosphorus fine chemicals. High temperature of the slag system for the smelting of phosphorite resulted in the energy-extensive consumption. Moreover, the low activity of slag led to the low utilization efficiency. In the current study, the effects of K2O addition on the reduction smelting of phosphorite were investigated via thermodynamic calculations and experiment validation in order to reduce the smelting temperature and increase the activity of the slag. It has been found that K2O could reduce the melting temperature of SiO2–CaO–Al2O3 ternary system and accelerate the liquid-phase formation. Higher phosphorus volatilization ratio over 97% was obtained when the phosphorite smelted at 1500 °C with binary basicity of 0.8 and 3 wt.% K2O addition . Moreover, a K-bearing slag with high activity was obtained and it can be used as micronutrient fertilizer.

Low-grade phosphate ores are the main phosphorus resources in China, and it’s difficult to upgrade owing to most ores belonging to collophanite with fine-grained dissemination. These inferior ores with high silicon and magnesium contents are favorable raw materials to produce fused magnesium phosphate fertilizer (FMP) for the crop growth. However, high temperature of Ca3(PO4)2–SiO2–MgO system for the smelting of phosphorite resulted in the energy-extensive consumption. In current study, the effect of K2O addition on the smelting of phosphorite was investigated via thermodynamic calculations and experiment validation to reduce the smelting temperature. It has been found that 3–5% K2O can reduce the melting temperature of the Ca3(PO4)2–SiO2–MgO ternary system by about 80 °C. Moreover, K2O addition can enhance the activities of various nutrients such as potassium and magnesium in the FMP products after smelting and water quenching, and the effective conversion of the nutrients is increased by 5% compared with that without K2O addition.

Pacing the cardiac conduction system has been explored in patients with conduction system disease, but comprehensive comparisons between different pacing modalities are not well investigated.To compare pacing characteristics and ventricular synchrony between His-bundle pacing (HBP) and left bundle branch pacing (LBBP) in patients with atrioventricular block (AVB).Fifty pacemaker-indicated patients with AVB were enrolled. Twenty-five patients underwent HBP, and another 25 patients underwent LBBP. Success rate, procedural and fluoroscopy duration, pacing parameters, and echocardiographic data were perioperatively assessed and at 3-month follow-up.HBP was successful in 19 of 25 (76.0%) patients, whereas LBBP was successful in 22 of 25 (88.0%) patients. Compared with HBP, LBBP capture threshold was significantly lower (0.76 ± 0.25 V/0.4 ms vs. 1.27 ± 0.61 V/1.0 ms, P = 0.003) and R-wave amplitude was significantly higher with LBBP (11.7 ± 6.6 vs. 4.9 ± 2.4 mV, P 0.001) at implant. The mean procedural time (74.3 ± 17.8 vs. 63.2 ± 12.3 min, P = 0.029) and fluoroscopy duration (10.3 ± 4.5 vs. 6.8 ± 2.2 min, P = 0.005) were significantly longer in the HBP group compared to LBBP. At 3-month follow-up, pacing capture threshold remained more stable in LBBP than in HBP group while left ventricular synchrony was similar between both groups.Despite similar impact on ventricular synchrony compared with HBP, LBBP featured a significantly lower pacing capture threshold, higher R-wave amplitude, and less time to achieve similar success rate in patients with AVB. These findings indicate LBBP as a physiological pacing strategy for AVB patients.

As a downstream interactor of β-catenin, Pangolin which is the homologous protein of the T cell factor/lymphoid enhancer factor (TCF/LEF) in vertebrates is less understood in the research field of immunity. In this study, two isoforms of Litopenaeus vannamei Pangolin (LvPangolin1 and LvPangolin2) were identified. Phylogenetic tree analysis revealed that all of the Pangolin proteins from invertebrates were represented the same lineage. The mRNA expression profiles of the LvPangolin1 and LvPangolin2 genes differed across different tissues. The expression of LvPangolin1 and the amount of LvPangolin1and LvPangolin2 combined (LvPangolinComb) were significantly increased in the haemocyte, intestine and gill but reduced in the hepatopancreas after white spot syndrome virus (WSSV) challenge. The inhibition of LvPangolin1 but not LvPangolinComb significantly reduced the survival rates of L. vannamei after WSSV infection, while significantly higher WSSV viral loads in both LvPangolin1-inhibited and LvPangolinComb-inhibited L. vannamei were observed. Knockdown of LvPangolin by RNAi could distinctly decrease the expression of antimicrobial peptide (AMP) genes and their related transcription factors. All of these results indicate that LvPangolin plays a positive role in the response to WSSV infection and that this may be mediated through regulating the immune signalling pathways which control the expression of AMPs with antiviral abilities.

High-P oolitic hematite is recognised as one of the most refractory mineral resources because of the special oolitic structure. Conventional selective reduction roasting is characterised by high energy consumption and large greenhouse gas emissions. In this study, a clean utilisation of refractory high-P oolitic hematite via phase reconstruction was proposed to make the P for crop fertiliser and Fe for ferrite ceramic. Phase reconstruction indicated that the oolitic and finely disseminated structure was dramatically changed by the oxidisation roasting with Mn ore at 1100°C–1250 °C. After reconstruction, 85 wt%–90 wt% Fe and Mn were concentrated in the magnetic ferrite phase whilst 90 wt%–100 wt% P, K, Ca, Si, Mg, and Al impurities were enriched in the nonmagnetic P-bearing tailings. After separation, the separated Mn ferrite and P-bearing tailings after activation could be used as ingredients in ceramic and fertiliser production. The as-purified ferrite showed good electromagnetism performance whilst almost all nutrients in the P-bearing tailings were activated via high-temperature melting and water quenching and can thus be readily absorbed by the crop roots. Moreover, the phase reconstruction mechanism and feasible granular fertiliser preparation from P-bearing tailings were discussed. All elements in the high-P oolitic hematite were utilised without emission of greenhouse gases.

Background Patient activity (PA) has been demonstrated to predict all-cause mortality. However, the association between PA and cardiac death is unclear. Aims The aims of this study were to determine whether PA can predict cardiac death and what is the cut-off of PA to discriminate cardiac death, as well as the mechanism underlying the relationship between PA and survival in patients with home monitoring. Methods This study retrospectively analysed clinical and implantable cardioverter-defibrillator/cardiac resynchronization therapy defibrillator device data in 845 patients. Data regarding PA and PP variability during the first 30-60 days of home monitoring were collected, and mean values were calculated. The primary endpoint was cardiac death, and the secondary endpoint was all-cause mortality. Results The mean PA percentage was 11 ± 5.8%. Based on receiver operating characteristic curve analysis, we determined that a PA cut-off value of 7.84% (113 min) can predict cardiac death. During a mean follow-up period of 31.1 ± 12.9 months (ranging from three to 60 months), PA ≤ 7.84% was associated with increased risks of cardiac death in an unadjusted analysis; after adjusting in a multivariate Cox model, the relationship remained significant between PA≤7.84% and cardiac death (hazard ratio = 3.644, 95% confidence interval = 2.424-5.477, p 0.001). Moreover, a significant correlation was observed between PA and PP variability ( r = 0.601, p 0.001). Conclusions A baseline PA ≤ 7.84% was associated with a higher risk of cardiac death in patients who have survived more than three months after implantable cardioverter-defibrillator/cardiac resynchronization therapy defibrillator implantation. PA had a sizable effect on heart rate variability, reflecting autonomic function.

Background Robust data regarding genotype–phenotype correlations in left ventricular noncompaction cardiomyopathy (LVNC) are lacking. Methods About 72 cardiomyopathy‐related genes were comprehensively screened in a cohort of LVNC patients using targeted sequencing. Baseline and follow‐up data were collected. The primary endpoint was a composite of death and heart transplantation. Results A total of 83 unrelated adult patients were included in analyses. Following stringent classification according to the American College of Medical Genetics and Genomics (ACMG) guidelines, 36 pathogenic variants of 14 genes were detected in 32 patients. Among them, 12 patients carried at least one nonsarcomere variant (NSV). At baseline, NSV carriers had a higher frequency of atrial fibrillation, but lower left ventricular ejection fraction, than did noncarriers. During a median follow‐up of 4.2 years, NSV carriers experienced a higher rate of the primary endpoint compared with noncarriers. There was no significant difference in the rate between carriers of sarcomere variant (SV) and noncarriers, as well as between carriers of SV and NSV. The presence of NSV was associated with an increased risk of the primary endpoint independent of age, sex, and cardiac function (hazard ratio: 3.61, 95% confidence interval: 1.42–9.19, p = .002). Conclusion NSV may act as a genetic modifier and worsen the clinical phenotype in patients with LVNC.

Nitrogen-doped mesoporous carbon spheres (NMCS) were prepared by a nanocasting route using benzoxazine resins as the precursor of nitrogen and carbon, and ordered mesoporous silica spheres as the hard template to remove methyl orange (MO) dye in aqueous solution. The prepared NMCS were respectively characterized by transmission electron microscopy, scanning electron microscopy, nitrogen sorption, X-ray diffraction, energy dispersive spectrometry, elemental analysis, thermal gravimetric analysis and X-ray photoelectron spectroscopy technologies. The characterization results show that NMCS are amorphous spherical nanoparticles with worm-like mesoporous channels, the specific surface area, pore volume and nitrogen contents were 634 m2 g−1, 0.91 cm3 g−1 and 3.50 (wt%), respectively. MO adsorption on NMCS was well fitted with the Langmuir adsorption isotherm and monolayer maximum adsorption capacity was up to 352.1 mg g−1 at 318 K, it was suggested that NMCS have excellent adsorption capacity for methyl orange. MO adsorption kinetics was found to follow the pseudo-second-order kinetic model, and the intra-particle diffusion model indicates that the rate-controlling step of adsorption was determined simultaneously by external mass transfer and intra-particle diffusion. The thermodynamic parameters indicated MO adsorption was a spontaneous, endothermic and feasible process. The free energy change was −27.79 kJ mol−1, −29.07 kJ mol−1 and −30.32 kJ mol−1 at 298 K, 308 K and 318 K, and the enthalpy and entropy change was 9.835 kJ mol−1 and 126.3 J (mol−1 K−1). Moreover, good regeneration properties and long life were observed for NMCS through the desorption experiments. MO adsorption should be mainly controlled by electrostatic attraction mechanism.

The first green revolution in 1960-1970 was a kind of visual sense green, obtain high yield of grain crops based on intensive production, high input and high output. While the high input of chemical fertilizer, inevitably accompanied by a huge consumption of chemical pesticides, and the large-scale intensive farming system damaged the soil structure, so it is an unsustainable agricultural development model. Facing the dual pressures of food security and resource environment protection, modern agriculture must achieve high output with high efficiency and resource environment protection, fertilizer development should improve fertilizer efficiency, improving quality instead of increasing quantity, green fertilizer ecological process must be innovated to improve the agriculture ecological environment. The contribution and shortage of the first green revolution was reviewed in this paper. Green fertilizer is a kind of environmental labeling, which maintaining ecological balance, protecting environment and human health. In order to make our country fertilizer consumption and environment pollution intensity quickly cross the top and then rapid reduction, it is necessary to establish a green fertilizer ensurance system, including raw materials, production process, product performance or characteristics of green fertilizer, green fertilization and ciuculation. It is pointed out that in the construction of the green fertilizer industrial system, the sustainable utilization of phosphorus resources especially the activation and high effective utilization of low grade phosphate rock and its mechanism study should be concerned; ecological fertilizer process with minimum entropy production, lowest energy consumption and environmental emissions should be focused on, some examples are given such as the service for family farm by using simple technology to provide green fertiizer, the preparation of small molecular carbon fertilizer by using industrial and agricultural wastes which is a kind of "dark green technology" combined with pollution control, ecological technology process of slow release fertilizer with low energy consumption and low emission, urea sulfuric acid compound fertilizer technology using low grade phosphate rock without phosphogypsum pollution and product containing multi nutrients with multi-function accordance with green chemistry principle of "atomic economy" etc.

Stochastic Brownian dynamics is an extremely powerful way to simulate the polymer dynamics in solutions and melts. Mathematically, these models are described by stochastic differential equations. The most challenging problems are the Monte Carlo algorithm, which simulates the motion of a large number of model particles and hence requires an enormous amount of computer time. It is therefore necessary to develop an efficient numerical method in operational emergency response applications. In this paper, we give an improved multilevel Monte Carlo (improved-MLMC) method based on equilibrium control variables at each level to calculate the propagation of polymers. The improved-MLMC method can be shown to result in asymptotically optimal random errors and reduce total cost when compared to the standard Monte Carlo and MLMC methods. Finally, the effect of the Wi number (dimensionless parameter) on the total cost of the presented MLMC method is also discussed in detail.

The polysulfide shuttling and serious volumetric change of sulfur during cycling are crucial obstacles to develop long-life Lithium–sulfur (Li–S) batteries. In this study, a novel sulfur host of SnO2-decorated MoS2 microboxes (MoS2@SnO2) was prepared by a simple template-assisted strategy and subsequent hydrothermal treatment. The results show that the synthesized MoS2@SnO2 composite exhibits a stable hollow porous structure, enlarged pore volume, increased surface area, and high binding energy by S–Sn–O chemical bonds, which remarkably improve physical and chemical dual-confined capability for polysulfides. Combined with exceptional structural stability and favorable electrolyte wettability, the MoS2@SnO2/S cathode demonstrates initial discharge capacity (1281 mAh g−1 at 0.2C) and superior long-cycling stability (701 mAh g−1 at 1C after 1000 cycles with an ultraslow capacity decay of ≈0.033% per cycle), which are much better than those of MoS2 microboxes/S and MoS2 microparticles/S cathodes. This work may provide a significant strategy for designing a multifaceted cathode by rationally integrating the relationships of structure, composition and property for long-life Li–S batteries.

Aims Two LMNA mutations (R644C and R190W) have been associated with familial and sporadic left ventricular non-compaction (LVNC). However, the mechanisms underlying these associations have not been elucidated. Methods and results Genomic DNA was isolated from peripheral blood leucocytes and analysed by direct sequencing. Human embryonic kidney 293 cells were transfected with either wild type or mutant LMNA and SCN5A for whole-cell patch-clamp experiment and fluorescence microscopy. Point mutation modeling for mutant LMNA was also performed. One novel LVNC-associated mutation (V445E) in β2 sheet of immunoglobulin (Ig)-like fold was found in the proband and his father. We also found that the peak current of sodium channel was markedly reduced in mutant LMNA compared with WT while the activation, inactivation, and recovery curves were not significantly altered. The mutant lamin A/C were aggregated into multiple highlighted particles. Three β sheets and multiple side chains in Ig-like fold were altered due to the replacement of a valine by glutamic acid. Conclusion Our data associated a novel lamin A/C mutation (V445E) with a sudden death form of familial LVNC. The reduced sodium current in mutant LMNA may account for the advent of malignant ventricular arrhythmias. The altered structures of three β sheets and side chains may partially explain the aggregation of lamin A/C protein subjacent to the nuclear envelope.

Although N-terminal pro-brain natriuretic peptide (NT-proBNP) is a useful screening test of impaired right ventricular (RV) function in conditions affecting the right-sided cardiac muscle, the role of NT-proBNP remains unclear in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). This study was designed to clarify the relation between the plasma NT-proBNP level and the RV function evaluated by cardiovascular magnetic resonance (CMR) imaging. We selected 56 patients with confirmed ARVC only when their blood specimens for NT-proBNP measurements were collected within 48 hours of a CMR scan. The NT-proBNP level was significantly higher in patients with RV dysfunction than in patients without RV dysfunction (median of 655.3 [interquartile range 556.4 to 870.0] vs 347.0 [interquartile range 308.0 to 456.2] pmol/L, p0.001). The NT-proBNP levels were positively correlated with RV end-diastolic and end-systolic volume indices (r = 0.49 and 0.70, respectively) and negatively correlated with RV ejection fraction (r = -0.76, all p0.001), which remained significant after adjustment for age, gender, and body mass index. The area under the receiver-operating characteristic curve for NT-proBNP was 0.91 (95% confidence interval 0.80 to 0.97, p0.001). The cut-off value of NT-proBNP (458 pmol/L) was associated with sensitivity, specificity, and positive and negative predictive values of 91%, 89%, 67%, and 98%, respectively. In conclusion, NT-proBNP is a useful marker for the detection of RV dysfunction and associated with extent of RV dilatation and dysfunction determined by CMR in patients with ARVC.

To compare cardiovascular magnetic resonance (CMR) characteristics between arrhythmogenic right ventricular cardiomyopathy (ARVC) patients with syncope and without syncope and explore CMR parameters related with syncope. A consecutive series of 80 patients with ARVC were divided in two groups according to history of syncope prior to CMR examinations. The biventricular function and volumes were calculated and indexed by body surface area. Fatty infiltration and late-gadolinium enhancement (LGE) were self-quantitatively analyzed according to segmental model. Patients with syncope had statistically significant greater left ventricular end-diastolic volume index (LVEDVI) (79.6 ± 23.0 vs. 69.0 ± 17.9 mL/m(2), P = 0.030), right ventricular end-diastolic volume index (RVEDVI) (122.0 ± 30.0 vs. 107.4 ± 21.8 mL/m(2), P = 0.017), and LGE incidence (52.2 vs. 21.1 %, P = 0.006) than that of patients without syncope. Patients with syncope had a trend towards greater number of segments with LGE (8.6 ± 4.2 vs. 6.6 ± 3.1, P = 0.199) than that of patients without syncope in subgroup analyses of patients with LGE, but no statistical significance was reached. Multivariate regression analysis showed the presence of LGE was independently associated with syncope in patients with ARVC (odds ratios 8.827, 95 % confidence interval 1.945-40.068, P = 0.005). CMR is helpful in detection and management of the patients with ARVC. Patients with syncope had significantly higher LVEDVI, RVEDVI and LGE incidence, and larger studies with follow-up data are needed to elucidate the relationship between LGE and syncope in patients with ARVC.

The genetic background of early repolarization syndrome (ERS) has not been fully understood. In this study, we identified a missense SCN5A mutation and a polymorphism in a patient with ERS and characterized the functional consequences of the two variants. The functional consequences of mutant channels were investigated with the patch-clamp technique, immunocytochemical studies and real-time PCR. A 19-year-old female proband with recurrent syncope had a documented electrocardiogram with ventricular fibrillation (VF) proceeded by large J waves in leads I, II, III, aVF and V2-V6. Genetic analysis revealed that the patient carried a missense mutation of c.4297 G>C and a synonymous polymorphism of T5457C on the same allele of the SCN5A gene. Patch-clamp experiments demonstrated that the c.4297 G>C mutation significantly reduced the sodium current (INa) density and altered the channel kinetics. Immunocytochemical studies demonstrated that the mutation dramatically inhibited the expression of sodium channels in the cell membrane and in the cytoplasm, although the mRNA levels remained in the normal range. Noteworthy, the reduction in INa density may be partially restored from the co-existence of the T5457C polymorphism on the same allele by the upregulation of mRNA levels. In conclusion, our study indicated that the c.4297 G>C mutation caused the 'loss-of-function' of sodium channels that may account for the clinical phenotype of ERS. The reduction in INa density was due to a decreased number of sodium channels caused by abnormal translation processes. The T5457C polymorphism partially rescued the INa density of the mutant channels by the upregulation of mRNA levels.

Aims. The molecular mechanisms of Chinese traditional medicine Wenxin Keli (WXKL) were unknown. This study was aimed at exploring the effects of WXKL on the gene expression profile and pathological alteration of rabbits with myocardial infarction.Methods. Twenty male adult rabbits were randomly divided into 4 groups: sham, model, WXKL, and captopril groups. Model, WXKL, and captopril groups underwent the ligation of the left anterior descending coronary artery while sham group went through an identical procedure without ligation. WXKL (817 mg/kg/d), captopril (8 mg/kg/d), and distilled water (to model and sham groups) were administered orally to each group. After 4 weeks, the rabbits were examined with echocardiography and the hearts were taken for expression chip and pathological staining (H&E, Masson, and Tunel) studies.Results. The data revealed that WXKL downregulated genes associated with inflammation (CX3CR1, MRC1, and FPR1), apoptosis (CTSC and TTC5), and neurohumoral system (ACE and EDN1) and upregulated angiogenesis promoting genes such as RSPO3. Moreover, the results also showed that WXKL improved cardiac function and prevented histopathological injury and apoptosis.Conclusion. The present study demonstrated that WXKL might play an important role in inhibiting inflammation, renin-angiotensin system, and apoptosis. It might be a promising Chinese medicine in the treatment of patients with myocardial infarction.

Several genetic variants have been associated with early repolarization syndrome (ERS). However, the lack of functional validations of the mutant effects has limited the interpretation of genetic tests. In the present study, we identified and characterized a novel sodium channel, voltage gated, type V alpha subunit (SCN5A) mutation that was associated with ERS. A 67-year-old male proband suffering from recurrent syncope underwent a documented electrocardiogram (ECG) for polymorphic ventricular tachycardia (VT). It was noted that baseline 12-lead ECG exhibited a predominantly elevated ST-segment which mimicked acute myocardial ischemia in lead V2–V6, and the ECG also demonstrated J waves in lead II, III, aVF and V2–V6. Using genetic analysis, we noted that the proband carried a novel heterozygous missense mutation of A1055G in the SCN5A gene. Whole-cell configuration of patch-clamp analysis revealed that the mutation significantly decreased peak sodium current (INa) density and shifted the steady-state inactivation curve of INa to a more negative potential. Confocal imaging suggested that in the mutant channel a defect of protein expression both on the cell membrane and in cytoplasm was present. The present study demonstrated that a novel heterozygous missense mutation of A1055G in SCN5A led to 'loss-of function' of the sodium channels, and we suggest that it accounts for the arrhythmogenic characteristics of ERS.

Background Although cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to CMR is conducted in children with LVNC. This study was performed to characterize and compare CMR features and clinical outcomes in children with LVNC with and without late gadolinium enhancement (LGE). Methods A cohort of 40 consecutive children (age, 13.7 ± 3.3 years; 29 boys and 11 girls) with isolated LVNC underwent a baseline CMR scan with subsequent clinical follow-up. Short-axis cine images were used to calculate left ventricular (LV) ejection fraction (EF), end-diastolic volume (EDV), end-systolic volume (ESV), myocardial mass, ratio of non-compacted-to-compacted myocardial thickness (NC/C ratio), and number of non-compacted segments. The LGE images were analyzed to assess visually presence and patterns of LGE. The primary end point was a composite of cardiac death and heart transplantation. Results The LGE was present in 10 (25 %) children, and 46 (27 %) segments were involved, including 23 non-compacted segments and 23 normal segments. Compared with LGE- cohort, LGE+ cohort had significantly lower LVEF (23.8 ± 10.7 % vs. 42.9 ± 16.7 %, p

Background Variants in NOS1AP associated with cardiac repolarization and sudden cardiac death (SCD) in coronary artery disease have been reported. Whether they are related to mortality and QTc interval in chronic heart failure (CHF) has not been investigated. Methods and Results A total of 1,428 patients with CHF and 480 control subjects were genotyped for 6 SNPs of NOS1AP, and the genetic associations with mortality as well as QTc interval were analyzed. During a median follow-up period of 52 months, 467 patients (32.70%) died, of which deaths 169 (36.19%) were SCD. The A allele of rs12567209 was associated with greater risk of all-cause death and SCD (hazard ratio [HR] 1.381, 95% confidence interval [CI] 1.124–1.698 [ P = .002], and HR 1.645, 95% CI 1.184–2.287 [ P = .003], respectively). After adjusting for other risk factors, significant differences remained (HR 1.309, 95% CI 1.054–1.624 [ P = .015], and HR 1.601, 95% CI 1.129–2.271 [ P = .008]). The A allele was also associated with prolongation of QTc interval by 4.04 ms in the entire population ( P = .026). Conclusions The A allele of rs12567209 in NOS1AP may serve as an independent predictor of all-cause death and SCD in patients with CHF, it is also associated with prolonged QTc interval in the Chinese Han population.