Novel IKBKG gene mutations in incontinentia pigmenti: report of two cases

Incontinentia pigmenti (IP), an X-chromosome dominant genodermatosis caused by mutations in the IKBKG/NEMO gene, is a rare disease affecting the skin, teeth, eyes, and central nervous system. Here, we report two pedigrees of IP and detection of two novel mutations in the IKBKG gene associated with IP via genetic analysis. In addition, different gene mutation types can present with different clinical phenotypes, and the same gene mutation type can show different clinical phenotypes. This study provides clinical cases for further study of the genotype and phenotype of IP and enriches the mutation spectrum of IKBKG gene, which provides a basis for genetic counseling and genetic diagnosis of IP in the future

PtdIns (3,4,5) P3 Recruitment of Myo10 Is Essential for Axon Development

Myosin X (Myo10) with pleckstrin homology (PH) domains is a motor protein acting in filopodium initiation and extension. However, its potential role has not been fully understood, especially in neuronal development. In the present study the preferential accumulation of Myo10 in axon tips has been revealed in primary culture of hippocampal neurons with the aid of immunofluorescence from anti-Myo10 antibody in combination with anti-Tuj1 antibody as specific marker. Knocking down Myo10 gene transcription impaired outgrowth of axon with loss of Tau-1-positive phenotype. Interestingly, inhibition of actin polymerization by cytochalasin D rescued the defect of axon outgrowth. Furthermore, ectopic expression of Myo10 with enhanced green fluorescence protein (EGFP) labeled Myo10 mutants induced multiple axon-like neurites in a motor-independent way. Mechanism studies demonstrated that the recruitment of Myo10 through its PH domain to phosphatidylinositol (3,4,5)-trisphosphate (PtdIns (3,4,5) P3) was essential for axon formation. In addition, in vivo studies confirmed that Myo10 was required for neuronal morphological transition during radial neuronal migration in the developmental neocortex

Evaluation of Tolerability, Pharmacokinetics and Pharmacodynamics of Vicagrel, a Novel P2Y12 Antagonist, in Healthy Chinese Volunteers

Background: Vicagrel is a novel anti-platelet drug and hydrolyzed to the same intermediate as clopidogrel via esterase, instead of CYP2C19. Here we report the first clinical trial on the tolerability, pharmacokinetics and pharmacodynamics of different doses of vicagrel, and comparison with clopidogrel in healthy Chinese volunteers.Methods: This study was conducted in two parts. Study I was a dose-escalating (5–15 mg) study. For each dose, 15 participants were randomized into three groups (total n = 45); nine participants were given vicagrel, three were given clopidogrel, and three were given a placebo. Study II was conducted to assess interactions between vicagrel and aspirin in 15 healthy participants. The plasma concentrations of the metabolites of vicagrel and clopidogrel were determined using a LC-MS/MS method. Platelet aggregation was assessed using the VerifyNow-P2Y12 assay.Results: Vicagrel (5–15 mg per day) dosing for 10 days or addition of aspirin was well tolerated in healthy volunteers. The exposure of the active metabolite increased proportionally across the dose range and was higher (~10-fold) than clopidogrel. The levels of IPA dosing 75 mg clopidogrel were between the responses of 5 mg and 10 mg vicagrel. After a single loading dose of vicagrel (30 mg) and a once-daily maintenance dose (7.5 mg) for 8 days, the maximum inhibition of platelet aggregation was similar to that seen with the combined use of vicagrel and aspirin (100 mg/day).Conclusion: Oral vicagrel demonstrated a favorable safety profile and excellent anti-platelet activity, which could be a promising P2Y12 antagonist as anti-platelet drug and can be further developed in phase II/III studies, and marketing for the unmet medical needs of cardiovascular diseases. The study was registered at http://www.chictr.org.cn (ChiCTR-IIR-16009260)

Covariation in Plant Functional Traits and Soil Fertility within Two Species-Rich Forests

The distribution of plant species along environmental gradients is expected to be predictable based on organismal function. Plant functional trait research has shown that trait values generally vary predictably along broad-scale climatic and soil gradients. This work has also demonstrated that at any one point along these gradients there is a large amount of interspecific trait variation. The present research proposes that this variation may be explained by the local-scale sorting of traits along soil fertility and acidity axes. Specifically, we predicted that trait values associated with high resource acquisition and growth rates would be found on soils that are more fertile and less acidic. We tested the expected relationships at the species-level and quadrat-level (20×20 m) using two large forest plots in Panama and China that contain over 450 species combined. Predicted relationships between leaf area and wood density and soil fertility were supported in some instances, but the majority of the predicted relationships were rejected. Alternative resource axes, such as light gradients, therefore likely play a larger role in determining the interspecific variability in plant functional traits in the two forests studied

Study on viral etiology of 942 cases of lower respiratory tract infections in Gansu Province during 2010-2013

Objective: To explore the common viruses and clinical features of lower respiratory tract infections(LRTIs) in Gansu Province.Methods: Through "infectious diseases monitoring technology platform information management system", 942 cases of LRTIs during 2010-2013 were collected from 10 sentinel hospitals in Gansu Province. Nasopharyngeal swabs, nasopharyngeal aspirates, sputum, bronchoalveolar lavage fluid, blood, serum and pleural puncture fluid were detected by polymerase chain reaction(PCR) for influenza(Flu),respiratory syncytial virus(RSV),adenovirus(PIV),human coronavirus (HCoV), adenovirus(Adv) and human metapneumovirus (h MPV). All of the information were analyzed by descriptive statistical methods.Results: The sex ratio was 1.93 ? 1 and the median age was 47.73. The detection rate of virus was 31.32% and the co-infections rate was 3.08%. The positive rates of Flu, RSV, PIV, HCo V, Adv and h MPV were 13.63%, 8.69%, 4.87%, 4.14%, 2.38% and 2.09%, respectively. RSV(20.00%), PIV(10.89%) and Flu (9.43%) were the main viruses in the group of 2 years old. Flu(15.35%), HCo V(7.09%) and RSV(5.71%) were the major viruses in the group of ? 15 years old. The major clinical syndrome included cough (89.81%), expectoration (64.65%) and fever (58.39%). Abdominal pain and diarrhea were 1.27% and 6.26%.Conclusions: The main viruses of LRIs in Gansu Province are Flu, RSV and PIV. The main viruses and manifestations of children and adults differ obviously.<br/

Metal-free photocatalyst of few-layer phosphorene with excellent activity under different light conditions

Herein, liquid exfoliation method was employed to prepare metal-free photocatalyst of few-layer phosphorene using black phosphorus as the precursor and its physicochemical properties were systematically characterized. The photocatalytic degradation experiments show that phosphorene exhibits more excellent photocatalytic activity toward tetracycline (TC) degradation under UV light irradiation with deoxidation. Moreover, the photodegradation rate constant of TC during the first 15 min of reaction is 0.2004 min(-1), which is 4.61 times of that under the pure UV light irradiation. Furthermore, the catalytic mechanism of phosphorene toward TC degradation was proposed and the excellent photocatalytic performance is mainly contributed to the efficient separation of photogenerated h(+) and e(-)

Biomass ash pyrolyzed from municipal sludge and its adsorption performance toward tetracycline: effect of pyrolysis temperature and KOH activation

Large amount of municipal sludge is difficult to handle; its resource utilization is an effective measure. In this study, the municipal sludge from sewage treatment plant was pyrolyzed without gas protection at different temperatures and potassium hydroxide (KOH) concentrations for activation. The pyrolysis products, named biomass ash, with higher surface area and enriched pore structures could be obtained at the pyrolysis temperature of 773 K. Moreover, the KOH activation for raw municipal sludge could further increase the surface area of the pyrolysis biomass ash. The maximum specific surface area was 44.71 m(2)/g, which was obtained under 2 mol/L KOH activation before pyrolysis at 773 K. And in this situation, the obtained pyrolysis biomass ash as adsorbent showed the maximum adsorption capacity of 50.75 mg/g toward tetracycline (TC). Moreover, the TC adsorption onto pyrolysis biomass ash obtained under various conditions followed the pseudo-second-order kinetic model. Adsorption thermodynamics analysis suggested the TC adsorption onto the pyrolysis biomass ash with no pre-activation was mainly due to the multi-molecule heterogeneous adsorption, while the TC adsorption onto pyrolysis biomass ash pretreated through the activation of KOH followed the monomer adsorption mechanism. This different adsorption mechanism was largely related to the pore structure, polarity, and aromaticity of the adsorbent

<p>Hydrothermal synthesis of dendritic CuBi2O4 and its photocatalytic performance towards tetracycline degradation under different light conditions</p>

Dendritic CuBi2O4 with excellent photocatalytic performance towards tetracycline (TC) degradation was firstly prepared by hydrothermal synthesis method. Light condition and oxygen atmosphere both play an important role in the photocatalysis of CuBi2O4. Under Mercury lamp irradiation with deoxidation, TC can be rapidly removed within initial 5 min and its photodegradation rate constant is 0.1247 min(-1), which is 36.68 times of that only under Mercury lamp irradiation. Moreover, free radical sequestration experiments suggest that the photo generated hole (h(+)) and electron (e(-)) are mainly involved in the degradation of TC. Besides, the efficient separation of h(+)-e(-) pairs contributes to the enhancement of photocatalytic activity of CuBi2O4

Novel visible-light-driven SrCoO3/Ag3PO4 heterojunction with enhanced photocatalytic performance for tetracycline degradation

The semiconductor photocatalytic technology has been considerably studied due to its excellent catalytic performance in water pollution control. Herein, in this study, novel SrCoO3/Ag3PO4 composite materials with different SrCoO3 content were synthesized via a simple hydrothermal synthesis method. The characteristics of the as-prepared samples were detected through SEM/HRTEM, XRD, UV-vis DRS, PL, ESR, FT-IR, and XPS techniques, and then, the photocatalytic performance of SrCoO3/Ag3PO4 toward the degradation of tetracycline was investigated. When the mass ratio of SrCoO3 and Ag3PO4 in the composite was 1:1.5, the degradation rate constant of tetracycline in SrCoO3/Ag3PO4 (1:1.5) system is 0.0102 min(-1), which is 1.7 times that of the Ag3PO4, and 3.78 times that of the SrCoO3. In addition, reactive species were also analyzed through the free radical trapping experiment and DMPO spin-trapping ESR spectra analysis, showing that OH center dot, h(+), and O(2)(center dot-)participated in the catalytic degradation process of tetracycline to varying degrees. Finally, the photocatalytic mechanism of SrCoO3/Ag3PO4 was also proposed

Dominant Impacting Factors on Water-Use Efficiency Variation in Inner Mongolia from 2001 to 2018: Vegetation or Climate?

Water-use efficiency (WUE) is not only an important indicator to connect the carbon and water cycles of a terrestrial ecosystem, but also a key parameter for an ecosystem to respond to climate change. It is crucial for understanding the mechanism of regional ecosystem response to environmental change by researching the influences of vegetation and climate change on WUE variation and revealing its drivers. Based on trend analysis, grey relational analysis, and ridge-regression analysis, this study analyzed the spatiotemporal variation characteristics of WUE in Inner Mongolia (IM) from 2001 to 2018 and determined the dominant influencing factors of WUE variation. The results showed that the annual mean WUE in IM was 1.39 g C m−2 mm−1 and it generally presented a rising trend, with an increasing rate of 0.0071 g C m−2 mm−1 yr−1. Leaf-area index (LAI) and precipitation were the most important factors influencing WUE in IM, followed by relative humidity and wind speed. Temperature, water vapor pressure and sunshine duration slightly influenced WUE and they were relatively less important. According to the ridge-regression analysis, LAI, precipitation and relative humidity had a positive contribution to WUE variation, while the wind speed had a negative contribution. Regionally, LAI was the dominant cause of WUE variation. The contribution and relative contribution rate of LAI to WUE variation were 0.008 g C m−2 mm−1 yr−1 and 44.57%, which were significantly higher than those of precipitation, relative humidity, and sunshine duration. Thus, vegetation primarily dominated WUE variability during the study period. The relative contribution rate of LAI varied across the different vegetation types and ranged from 25.26% in swamps to 51.29% in meadows. Our results improve the understanding of the effects of driving factors on WUE, which can help policymakers with water resource management and ecological restoration