SUMMARY Here, we report the clinical observations of two Chinese fraternal twins who presented with severe dehydration, poor feeding, and absence of stimuli responses within a few days of birth. Trio clinical exome sequencing of the family identified compound heterozygous intronic variants (c.1439+1G>C and c.875+1G>A) in SCNN1A gene in these two patients. Sanger sequencing results showed that the c.1439+1G>C variant was inherited from the mother, and c.875+1G>A from the father, rarely reported in pseudohypoaldosteronism type 1 with sodium epithelial channel destruction (PHA1b) patients. Case 2 received timely symptomatic treatment and management after obtaining these results, which improved the clinical crisis. Our results suggest that the compound heterozygous splicing variants in SCNN1A were responsible for PHA1b in these Chinese fraternal twins. This finding extends the knowledge of the variant spectrum in PHA1b patients and highlights the application of exome sequencing in critically ill newborns. Finally, we discuss supportive case management, particularly in maintaining blood potassium concentration. Here dehydration feeding birth c14391GC cGC c 1439 1G C G c8751GA cGA 875 A SCNNA SCNN mother father PHAb PHA b (PHA1b crisis newborns Finally concentration GC 143 GA 87 14 8

ABSTRACT Atopopompilus Arnold, 1937 is a small genus of Pompilini. Most species are distributed in the Afrotropical Region, but there are a few in the Oriental region. In this contribution, the taxonomy of Atopopompilus from China is addressed, to ascertain species diversity and distribution in the country. One new species, Atopopompilus robusticarinatus Yang & Ma, sp. nov., is described from Yunnan and Tibet. The new species is similar to Atopopompilus daedalus daedalus (Bingham, 1896), but can be distinguished by the facial carina, the posterior margin of the pronotum and the male SGP and genitalia. In addition, the geographic distribution of and a key to the world species of Atopopompilus are updated. This study increased the number of known species Atopopompilus, and expanded the known distribution of the genus to the Palearctic region. Arnold 193 Pompilini Region region contribution addressed country Ma sp nov nov. Tibet Bingham, Bingham (Bingham 1896, 1896 , 1896) carina genitalia addition updated 19 189 1 18

Abstract Microbial fermentation can be responsible for flavor production and increasing the nutritional quality of fermented fish foods. The study aimed to isolate non-halophilic bacteria possessing proteolysis and lipolysis properties as starter cultures for low-salt content fermentation of coarse fish. The strains with high enzyme activities were screened and multiple growth characteristics were analyzed. The content of amino acid nitrogen and trichloroacetic acid (TCA)-soluble peptides in fermented fish was also investigated under different fermentation conditions. Nuclear magnetic resonance (NMR) metabolomics analysis was utilized to monitor the metabolite profile of fermented fish paste. The isolate was identified as Bacillus velezensis DZ11. The strain DZ11 exhibited obvious hydrolysis properties of lipids and proteins. During the fermentation of fish paste, a total of 40 differential metabolites were identified, of which multiple metabolites changed significantly, including nutrient-related substances and flavor precursors. Salt concentration and strain DZ11 had important effects on the differential metabolites. B. velezensis DZ11 under longer fermentation time promoted the formation of flavor precursors and potentially increased the nutritional substances of low-salt fermented fish. foods nonhalophilic non halophilic lowsalt low salt analyzed TCAsoluble TCA soluble conditions NMR (NMR paste DZ DZ1 proteins 4 significantly nutrientrelated nutrient related B

BACKGROUND: Scholars have examined populations within firms and found that managers and employees exhibit similar characteristics in the relationship between dynamic capability drivers and sustainable performance. However, the unobserved relationship between dynamic capability drivers and sustainable performance in the context of dairy microfirms is less investigated AIM: The main motive is to examine the unobserved connection in the relationship between dynamic capability drivers and sustainable performance in dairy microfirms in Tanzania. Illustrating the knowledge-based view (KBV), this study determines that valuable competencies impact dairy microfirms' sustainable performance SETTING: The 300 participants in this study were employees and managers of dairy microfirms in three regions of Tanzania: Tanga, Arusha, and Kilimanjaro METHOD: A unique unit segment technique - response-based unit segmentation-partial least squares (REBUS-PLS) path modelling - is used to uncover latent classes to meet the research objective RESULTS: Our findings reveal that the aggregate model hypotheses were significant. Furthermore, the paper illuminates potential unobserved variations between managers and employees concerning the dynamic capability drivers and sustainable performance of dairy microfirms in Tanzania CONCLUSION: The potential unobserved differences between managers and employees provide an alternative explanation for the relationship between dynamic capability drivers and sustainable performance. This helps avoid the 'competency trap' and explains how to improve the dynamic capabilities of dairy microfirms CONTRIBUTION: Homogeneous behaviour among managers and employees strongly suggests collectivist work to improve sustainable performance. We contribute empirically by demonstrating the underlying dynamic capability drivers of managers and employees in heterogeneous segments to explain sustainable performance

Abstract Objective To explore the molecular mechanism of neuropathologic damage induced by radiofrequency ablation at different temperatures. Methods This is basic research, and 36 SD rats were used to construct the neuropathological injury model. The rats were subjected to radiofrequency stimulation at different temperatures and were divided into 6 groups according to the temperature injury: 42°, 47°, 52°, 57°, 62°, and 67°C groups. Conduction time, conduction distance, and nerve conduction velocity were recorded after temperature injury. HE-staining was used to observe the histopathological morphology of the sciatic nerve. The expression of SCN9A, SCN3B, and NFASC protein in sciatic nerve tissue were detected by western blot. Results With the increase in temperature, nerve conduction velocity gradually decreased, and neurons were damaged when the temperature was 67°C. HE-staining showed that the degrees of degeneration of neurons in rats at 47°, 52°, 57°, 62°, and 67°C were gradually increased. The expression of SCN9A, SCN3B protein in 57°, 62°, 67°C groups were much higher than that of NC, 42°, 47°, 52°C groups. However, the expression of NFASC protein in 57°, 62°, 67°C groups was much lower than that of the NC, 42°, 47°, 52°C groups. Conclusion There was a positive correlation between temperature caused by the radiofrequency stimulation to neuropathological damage. The mechanism is closely related to the expression of SCN9A, SCN3B, and NFASC protein in nerve tissue caused by heat transfer injury.

ABSTRACT A new species, Spilomena capatrata Bashir & Ma, sp. nov. is described from Palearctic and Oriental China. Additionally, eleven species are reported, of which five are new records from China: S. beata Blüthgen, S. hainesi N. Smith, S. menkei R. Bohart, S. punctatissima Blüthgen, and S. valkeilai Vikberg. A key to Chinese species of Spilomena Shuckard is also provided.

This study aimed to explore the effect of microRNA (miR)-146a inhibition on regulating cell apoptosis, total neurite outgrowth, inflammation, and STAT1/MYC pathway in Alzheimer's disease (AD). PC12 and cortical neuron cellular AD models were constructed by Aβ1-42 insult. For the former model, nerve growth factor (NGF) stimulation was previously conducted. miR-146a inhibitor and negative-control (NC) inhibitor were transfected into the two cellular AD models, and then cells were named miR-inhibitor group and NC-inhibitor group, respectively. After transfection, cell apoptosis, total neurite outgrowth, supernatant inflammation cytokines, and STAT1/MYC pathway were detected. miR-146a expression was similar between PC12 cellular AD model and control cells (NGF-stimulated PC12 cells), while miR-146a expression was increased in cortical neuron cellular AD model compared with control cells (rat embryo primary cortical neurons). In both PC12 and cortical neuron cellular AD models, miR-146a expression was reduced in miR-inhibitor group compared with NC-inhibitor group after transfection. Furthermore, cell apoptosis was attenuated, while total neurite outgrowth was elevated in miR-inhibitor group compared with NC-inhibitor group. As for supernatant inflammatory cytokines, tumor necrosis factor-α, interleukin (IL)-1β, IL-6, and IL-17 levels were lower in miR-inhibitor group than in NC-inhibitor group. Additionally, STAT1 and c-Myc mRNA and protein expressions were attenuated in miR-inhibitor group compared with NC-inhibitor group. In conclusion, miR-146a potentially represented a viable therapeutic target for AD.

Abstract Pain is a significant problem worldwide that affects the quality of life of patients. Dezocine is a non-addictive analgesic drug with kappa-opioid antagonist activity and has been successfully used to alleviate of postoperative pain. In addition, dezocine has an analgesic effect similar to that of morphine, alleviating moderate to severe pain. Rap guanine nucleotide exchange factor 3 (RAPGEF3) is a guanine nucleotide exchange factor for GTPases Rap1 and Rap2, which could enhance the activity of Rap1 to promote cell adhesion and axon regeneration, as well as promote neurite extension by interacting with nerve growth factors. Here, we first observed that overexpression of RAPGEF3 increased cell viability, as shown by a CCK-8 assay, and recovered brain function in rats. The expression of inflammation-related factors at the mRNA level was detected using qPCR, and the concentration of these factors in a cultured cell medium and rat serum samples were decreased as shown by ELISA after RAPGEF3 overexpression. Through western blotting, we further found that pro-inflammatory proteins were decreased, and these effects might be mediated by inhibition of the Ras/p-38 MAPK signaling pathway. Taken together, we speculated that RAPGEF3overexpression enhances the therapeutic effect of dezocine on neuropathic pain by inhibiting the inflammatory response through inhibition of the Ras/p-38 MAPK signaling pathway.

Abstract Background: Basal-like breast cancer (BLBC) or triple-negative breast cancer (TNBC) is an aggressive and highly metastatic subtype of human breast cancer. The present study aimed to elucidate the potential tumor-suppressive function of MATR3, an abundant nuclear protein, in BLBC/TNBC, whose cancer-relevance has not been characterized. Methods: We analyzed in vitro tumorigenecity by cell proliferation and soft agar colony formation assays, apoptotic cell death by flow cytometry and Poly (ADP-ribose) polymerase (PARP) cleavage, epithelial-mesenchymal transition (EMT) by checking specific EMT markers with real-time quantitative PCR and in vitro migration and invasion by Boyden Chamber assays. To elucidate the underlying mechanism by which MATR3 functions as a tumor suppressor, we performed Tandem affinity purification followed by mass spectrometry (TAP-MS) and pathway analysis. We also scrutinized MATR3 expression levels in the different subtypes of human breast cancer and the correlation between MATR3 expression and patient survival by bioinformatic analyses of publicly available transcriptome datasets. Results: MATR3 suppressed in vitro tumorigenecity, promoted apoptotic cell death and inhibited EMT, migration, and invasion in BLBC/TNBC cells. Various proteins regulating apoptosis were identified as MATR3-binding proteins, and YAP/TAZ pathway was suppressed by MATR3. MATR3 expression was inversely correlated with the aggressive and metastatic nature of breast cancer. Moreover, high expression levels of MATR3 were associated with a good prognosis of breast cancer patients. Conclusions: Our data demonstrate that MATR3 functions as a putative tumor suppressor in BLBC/TNBC cells. Also, MATR3 potentially plays a role as a biomarker in predicting chemotherapy-sensitivity and patient survival in breast cancer patients.

Resumo Fundamento: Níveis plasmáticos elevados de lipoproteína (a) [Lp(a)] são reconhecidos como um fator de risco significativo para doença vascular aterosclerótica. No entanto, existem dados limitados sobre a associação entre a Lp(a) e insuficiência cardíaca (IC) recorrente em pacientes com IC crônica causada por doença arterial coronariana (DAC). Objetivo: Níveis elevados de Lp(a) podem ter um impacto prognóstico na IC recorrente em pacientes com IC crônica por DAC. Métodos: Um total de 309 pacientes com IC crônica causada por DAC foram consecutivamente incluídos neste estudo. Os pacientes foram divididos em 2 grupos de acordo com os níveis de Lp(a), acima ou abaixo do nível mediano de toda a coorte (20,6 mg/dL): o grupo Lp(a) alto (n = 155) e o grupo Lp ( a) baixo (n = 154). Um p < 0,05 bicaudal foi considerado estatisticamente significativo. Resultados: Durante a mediana do período de seguimento de 186 dias, 31 casos de um total de 309 pacientes (10,03%) não puderam ser contatados durante o acompanhamento. A análise de Kaplan-Meier demonstrou que pacientes com níveis mais elevados de Lp(a) apresentavam maior incidência de IC recorrente do que aqueles com níveis mais baixos de Lp(a) (log-rank < 0,0001). Uma análise de regressão multivariada de Cox revelou que os níveis de Lp(a) foram independentemente correlacionados com a incidência de IC recorrente após ajuste de potenciais fatores de confusão (hazard ratio 2,720, intervalo de confiança de 95%: 1,730-4,277, p < 0,0001). Conclusões: Em pacientes chineses com IC crônica causada por DAC, níveis elevados de Lp(a) estão associados de forma independente à IC recorrente.

Abstract Background: Elevated plasma levels of Lipoprotein(a) [Lp(a)] are recognized as a significant risk factor for atherosclerotic vascular disease. However, there are limited data regarding association between Lp(a) and recurrent heart failure (HF) in patients with chronic HF caused by coronary heart disease (CHD). Objective: Elevated levels of Lp(a) might have a prognostic impact on recurrent HF in patients with chronic HF caused by CHD. Methods: A total of 309 patients with chronic HF caused by CHD were consecutively enrolled in this study. The patients were divided into 2 groups according to whether Lp(a) levels were above or below the median level for the entire cohort (20.6 mg/dL): the high Lp(a) group (n = 155) and the low Lp(a) group (n = 154). A 2-sided p < 0.05 was statistically considered significant. Results: During the median follow-up period of 186 days, 31 cases out of a total of 309 patients (10.03%) could not be reached during follow-up. A Kaplan-Meier analysis demonstrated that patients with higher Lp(a) levels had a higher incidence of recurrent HF than those with lower Lp(a) levels (log-rank < 0.0001). A multivariate Cox regression analysis revealed that Lp(a) levels were independently correlated with the incidence of recurrent HF after adjustment of potential confounders (hazard ratio: 2.720, 95 % confidence interval: 1.730-4.277, p < 0.0001). Conclusions: In Chinese patients with chronic HF caused by CHD, elevated levels of Lp(a) are independently associated with recurrent HF.

The reaction mechanism of AgIII complex ([Ag(HIO6)2]5–) with ruthenium(II) bipyridine (Ru(bpy)32+) in acid medium was investigated. A novel flow injection chemiluminescence (CL) analysis method was developed for the detection of norfloxacin (NFLX) and ofloxacin (OFLX) in commercial drug, milk and human urine samples based on AgIII-ruthenium(II) bipyridine in acidic solution. Under optimal conditions, CL intensities were proportional to drug concentrations in the range of 1.6 × 10-9 to 8 × 10-6 g mL-1 for NFLX and 1.0 × 10-9 to 4 × 10-6 g mL-1 for OFLX. The detection limit was 1.0 × 10-9 g mL-1 for NFLX and 0.8 × 10-9 g mL-1 for OFLX. On the basis of the CL, UV and fluorescence spectra, possible reaction mechanisms were proposed.

Abstract Objective: To investigate the clinical significance of serum cystatin C (Cys-C) and high-sensitivity C-reactive protein (hs-CRP) in coronary heart disease (CHD) patients undergoing percutaneous coronary intervention (PCI). Methods: One hundred and twenty-eight CHD patients were divided into drug treatment (56 cases) and PCI treatment (72 cases) groups, receiving conventional drug treatment and PCI plus conventional drug treatment, respectively. At admission time and 4 weeks after treatment, the left ventricular ejection fraction (LVEF), left ventricular end diastolic diameter, and left ventricular end systolic diameter were measured. At admission time and 24h, 72h, 1 week, and 4 weeks after treatment, the serum levels of Cys-C and hs-CRP were determined. Results: After 4 weeks of treatment, LVEF in the PCI treatment group was significantly higher than that before treatment (P<0.01) and it was significantly higher than in the drug treatment group at the same time (P<0.01). Cys-C and hs-CRP level in the PCI treatment group were significantly higher than in the drug treatment group 72h and 1 week after treatment (P<0.05 or P<0.01), respectively, but they were significantly lower than in the drug treatment group 4 weeks after treatment (P<0.01). There were obvious interaction effects between grouping factor and time factor in Cys-C (F=3.62, P<0.05) and hs-CRP (F=17.85, P<0.01). Conclusion: Serum levels of Cys-C and hs-CRP are closely related to the heart function in CHD patients undergoing PCI, and they may be used for predicting the outcome of PCI.

Droplet atomization characteristics and its morphology in twin-wire arc sprayed (TWAS) Ni-Al were explored in depth. Both numerical model and theoretical method were built for calculating the process of the droplet deformation and breakup in air flow, which based on the standard k-ε model and the volume of fluid (VOF) dual-phase flow model. The atomization characteristics of double droplets and three droplets were also studied in the atomization process by simulation. The micro structure and the morphology of the composite coatings were analyzed by SEM and TEM. The experimental results indicate that TWAS droplets are the process of explosive breakup or two steps breakup. Morphology of the TWAS prepared Ni-20wt.%Al and Ni-5wt.%Al particles was accordingly characterized by SEM. The phase compositions of the Ni-Al particles were obtained by EDS. By testing the velocity of sprayed particles, the change rule of particles with spray pressure was obtained. Ni twins are discovered in the Ni-Al coating due to rapid cooling in TWAS process.

Abstract Purpose To investigate the effect of sevoflurane preconditioning on ischemia/reperfusion (I/R)-induced pulmonary/hepatic injury Methods Fifty-one Wistar rats were randomly grouped into sham, I/R, and sevoflurane groups. After reperfusion, the structural change of the lung was measured by Smith score, the wet and dry weights (W/D) were determined, malondialdehyde (MDA) myeloperoxidase (MPO) content was determined colorimetrically and by fluorescence, respectively, and matrix metalloprotein-9 (MMP-9) mRNA was quantified by RT-PCR. Biopsy and morphological analyses were performed on liver tissue, activities of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were determined, and tumor necrosis factor-alpha (TNF-α) level was determined. Results The sham group showed no changes in tissue structure. Structural lesions in the sevoflurane and I/R groups were mild and severe, respectively. Smith score, W/D, MDA, MPO, and MMP mRNA showed the same trend, and were increased in the I/R group and recovered in the sevoflurane group, compared with the sham group (both P<0.05). AST and ALT were significantly increased compared to the sham group (AST: 655±52.06 vs . 29±9.30 U/L; ALT: 693±75.56 vs . 37±6.71 U/L; P<0.05). In the sevoflurane group, AST and ALT levels were significantly decreased (464±47.71 and 516±78.84 U/L; P<0.001). TNF-α presented similar results. Conclusion The protection of lung and liver by sevoflurane may be mediated by inhibited leukocyte recruitment and MMP-9 secretion.

Droplets deformation and solidification characteristics in twin-wire arc sprayed Ni-Al were explored in depth. Both theoretical method and numerical model were established for calculating the deformation process of the droplets impacting on the substrate and their solidification behavior in airflow, which based on two models of the volume of fluid (VOF) dual-phase flow and the standard k-ε. The morphology and the microstructure of the composite coatings were analyzed by XRD, SEM and TEM. The results of the experiment indicate the droplets cooling rate ranges from 3.0×107 to 7.5×107 K/s. The main components of Ni-5wt.% Al coating were Ni solid solution, and a small number of Ni3Al4, Al2O3 and NiO. The main phases of Ni-20wt.% Al coating are Ni3Al and NiAl. EDS and TEM analysis shows that there are a few amorphous and equiaxed crystals in the Ni-Al coating. The surface roughness increases with the decrease of spraying pressure and spraying distance.