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 In order to explore the efficient investigate and utilization of emulsion produced by enzyme-assisted aqueous extraction of walnut oil, the effects of different enzyme treatment (including Alcalase 2.4L, Ban 480L α-amylase, Neutrase 0.8L, Pectinex SP-L, Celluclast 1.5L) on the characteristics of emulsion were studied to characterize its emulsifying characteristics The emulsion composition, particle size distribution, Zeta potential, rheological properties and protein surface hydrophobicity of emulsion were determined to characterize its emulsifying properties. The results showed that the particle size of the emulsion obtained from enzymatic hydrolysis showed a smaller trend and the absolute value of Zeta potential showed a larger trend in the presence of enzymes. On emulsion protein, hydrophobicity of emulsion protein was obtained by hydrolysis with different enzymes showed that the trend followed the order: Celluclast 1.5L> Ban 480L >Neutrase 0.8L >Pectinex SP-L >control> Alcalase 2.4L. The particle size, zeta potential, ability to increase emulsion viscosity and interfacial hydrophobicity of emulsion proteins affected the emulsion physical stability and the effect of demulsification. The results of rheological analysis show that emulsions from Ban 480L α-amylase, Neutrase 0.8L and Celluclast 1.5L hydrolysate still maintained their elastic structure damaged by shear rate and were more stable, and the emulsions obtained by Alcalase 2.4L and Pectinex SP-L were unstable which was easy to be broken. enzymeassisted assisted oil including 24L L 2 4L αamylase, αamylase α amylase, amylase α-amylase 08L 0 8L SPL, SPL SP L, 15L 1 5L composition distribution control >control demulsification stable broken

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.

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.

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.

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.

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 This study aimed to investigate the roles of miRNA in the development of ovarian follicles in sows with high ovulation rate. The miRNA expression profiles in middle-size ovarian follicles between Meishan and Duroc sows were characterized by Affymetrix miRNA GeneChip. Seven differentially expressed miRNA were identified. KEGG pathways analyzed the predicted target genes of differentially expressed miRNA by using the DAVID website. The MAPK signaling pathway may affect the process of ovarian follicle development. Our study suggested that miR-21, miR-199a, miR-708, and miR-4334 might play a vital role in the development of ovarian follicles of sow.

Abstract Purpose: To investigate the efficacy and mechanisms of root tuber of Polygonum ciliinerve (Nakai) ohwi (rPC) which has been used to treat bacterial infection in traditional Chinese medicine. Methods: With the mouse model of Staphylococcus aureus (S. aureus) pneumonia, the phenotype of rPC treated mice, including body weight, mortality, lung slices and bacterial burden were evaluated. Furthermore, inflammatory factors in bronchoalveolar lavage (BAL) were determined by ELISA and the distribution of T cells in lung was assessed by immunofluorescence assay. Results: rPC treatment could dose-dependently reduce weight loss and mortality in S. aureus-infected mice. Upon 10 mg/ml rPC treatment, S. aureus-infected mice showed about 8 grams increase in body weight (P<0.001) and 50% enhancement in mortality. The integrity of lung tissue and bacterial burden were also improved by rPC treatment. Moreover, rPC was found to modulate the immune response in infection. Conclusion: rPC has therapeutic potential for S. aureus infections and pneumonia with immunomodulatory functions.

There are enormous resources of unconventional gas in coal measures in Ordos Basin. In order to study the geological characteristics of unconventional gas in coal Measures in Ordos Basin, we analyzed and summarized the results of previous studies. Analysis results are found that, the unconventional gas in coal measures is mainly developed in Upper Paleozoic in Eastern Ordos Basin, which including coalbed methane, shale gas and tight sandstone gas. The oil and gas show active in coal, shale and tight sandstone of Upper Paleozoic in Ordos Basin. Coalbed methane reservoir and shale gas reservoir in coal measures belong to "self-generation and self-preservation", whereas the coal measures tight sandstone gas reservoir belongs to "allogenic and self-preservation". The forming factors of the three different kinds of gasses reservoir are closely related and uniform. We have the concluded that it will be more scientific and reasonable that the geological reservoir-forming processes of three different kinds of unconventional gas of coal measures are studied as a whole in Ordos Basin, and at a later stage, the research on joint exploration and co-mining for the three types of gasses ought to be carried out.

Background The Tibetan pig is a pig breed with excellent grazing characteristics indigenous to the Qinghai-Tibet plateau in China. Under conditions of barn feeding, 90% of its diet consists of forage grass, which helps meet its nutritional needs. The present study aimed to isolate and identify a cellulolytic bacterium from the Tibetan pig's intestine and investigate cellulase production by this bacterium. The study purpose is to provide a basic theory for the research and development of herbivore characteristics and to identify a source of probiotics from the Tibetan pig. Results A cellulolytic bacterium was isolated from a Tibetan pig's intestine and identified based on morphological, physiological, and biochemical characteristics as well as 16S rRNA analysis; it was designated Bacillus subtilis BY-2. Examination of its growth characteristics showed that its growth curve entered the logarithmic phase after 8-12 h and the stable growth phase being between 20 and 40 h. The best carbon source for fermentation was 1% corn flour, while 2% peptone and yeast powder compound were the best nitrogen sources. The initial pH during fermentation was 5.5, with 4% inoculum, resulting in a high and stable amount of enzyme in 24-48 h. Conclusions The isolated BY-2 strain rapidly grew and produced cellulase. We believe that BY-2 cellulase can help overcome the shortage of endogenous animal cellulase, improve the utilization rate of roughage, and provide strain sources for research on porcine probiotics.

Um método simples e sensível baseado na análise de quimiluminescência por injeção em fluxo acoplado ao complexo luminol-Ag(III) foi desenvolvido para se determinar ácido úrico (UA) em urina humana. O método se baseia na reação de quimiluminescência (CL) do luminol com Ag(III) em solução alcalina, sendo que o UA da urina aumenta dramaticamente as intensidades CL. Em condições ótimas, a intensidade CL relativa aumentava linearmente com a concentração de UA, na faixa de 1.0 × 10-8 a 5.0 × 10-6 mol L-1 com um limite de detecção de 2.0 × 10-9 mol L-1 (3σ). O desvio padrão relativo (n = 11) era de 0.71% para 1.0 × 10-7 mol L-1 UA. Os mecanismos de reação possíveis do sistema CL foram sugeridos com base nos espectros de absorção UV, espectros CL e no experimento de trapping de radicais livres feitos neste trabalho.

A simple and sensitive method based on flow-injection chemiluminescence analysis coupled with luminol-Ag(III) complex was developed for the determination of uric acid (UA) in human urine. The method was based on the chemiluminescence (CL) reaction of luminol with Ag(III) in alkaline solution. UA in the urine can dramatically enhance CL intensities. In optimum conditions, the relative CL intensity had a linear relationship with UA concentration in the range of 1.0 × 10-8 to 5.0 × 10-6 mol L-1 with a detection limit of 2.0 × 10-9 mol L-1 (3σ). The relative standard deviation (n = 11) was 0.71% for 1.0 × 10-7 mol L-1 UA. Possible reaction mechanisms for the CL system were suggested based on the UV absorption spectra, CL spectra, and the free radical trapping experiment performed in this work.

A leptina, uma adipocina produzida principalmente pelo tecido adiposo branco, tem um papel importante não somente na regulação da ingestão alimentar, mas também no controle da imunidade e da inflamação. Já foi amplamente demonstrado que a ausência de leptina causa deficiências imunológicas em modelos animais e em humanos, levando ao aumento da mortalidade. A leptina também regula a inflamação por meio da ação em seu receptor, amplamente distribuído em diversos tipos de células do sistema imunológico. Os mecanismos moleculares pelos quais a leptina determina suas ações biológicas foram recentemente elucidados, e três cascatas intracelulares são ativadas pela leptina: JAK-STAT, PI3K e ERK 1/2. Essas cascatas são reguladas por proteínas intracelulares, reduzindo as ações da leptina. Nesta revisão, são discutidos os mecanismos moleculares pelos quais a leptina regula a imunidade e a inflamação, associando-os a enfermidades inflamatórias crônicas. Arq Bras Endocrinol Metab. 2012;56(9):597-607

Leptin, the adipokine produced mainly by the white adipose tissue, plays important roles not only in the regulation of food intake, but also in controlling immunity and inflammation. It has been widely demonstrated that the absence of leptin leads to immune defects in animal and human models, ultimately increasing mortality. Leptin also regulates inflammation by means of actions on its receptor, that is widely spread across different immune cell populations. The molecular mechanisms by which leptin determines its biological actions have also been recently elucidated, and three intracellular pathways have been implicated in leptin actions: JAK-STAT, PI3K, and ERK 1/2. These pathways are closely regulated by intracellular proteins that decrease leptin biological activity. In this review, we discuss the molecular mechanisms by which leptin regulates immunity and inflammation, and associate those mechanisms with chronic inflammatory disorders. Arq Bras Endocrinol Metab. 2012;56(9):597-607