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NASH (non-alcoholic steatohepatitis) is a severe liver disease characterized by hepatic chronic inflammation that can be associated with the gut microbiota. In this study, we explored the therapeutic effect of Gynostemma pentaphyllum extract (GPE), a Chinese herbal extract, on methionine- and choline-deficient (MCD) diet-induced NASH mice. Based on the peak area, the top ten compounds in GPE were hydroxylinolenic acid, rutin, hydroxylinoleic acid, vanillic acid, methyl vanillate, quercetin, pheophorbide A, protocatechuic acid, aurantiamide acetate, and iso-rhamnetin. We found that four weeks of GPE treatment alleviated hepatic confluent zone inflammation, hepatocyte lipid accumulation, and lipid peroxidation in the mouse model. According to the 16S rRNA gene V3-V4 region sequencing of the colonic contents, the gut microbiota structure of the mice was significantly changed after GPE supplementation. Especially, GPE enriched the abundance of potentially beneficial bacteria such as Akkerrmansia and decreased the abundance of opportunistic pathogens such as Klebsiella. Moreover, RNA sequencing revealed that the GPE group showed an anti-inflammatory liver characterized by the repression of the NF-kappa B signaling pathway compared with the MCD group. Ingenuity Pathway Analysis (IPA) also showed that GPE downregulated the pathogen-induced cytokine storm pathway, which was associated with inflammation. A high dose of GPE (HGPE) significantly downregulated the expression levels of the tumor necrosis factor-α (TNF-α), myeloid differentiation factor 88 (Myd88), cluster of differentiation 14 (CD14), and Toll-like receptor 4 (TLR4) genes, as verified by real-time quantitative PCR (RT-qPCR). Our results suggested that the therapeutic potential of GPE for NASH mice may be related to improvements in the intestinal microenvironment and a reduction in liver inflammation.
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Microbioma Gastrointestinal , Gynostemma , Hepatopatia Gordurosa não Alcoólica , Extratos Vegetais , Animais , Microbioma Gastrointestinal/efeitos dos fármacos , Hepatopatia Gordurosa não Alcoólica/tratamento farmacológico , Camundongos , Gynostemma/química , Extratos Vegetais/farmacologia , Masculino , Inflamação/tratamento farmacológico , Fígado/efeitos dos fármacos , Fígado/metabolismo , Camundongos Endogâmicos C57BL , Modelos Animais de Doenças , Transdução de Sinais/efeitos dos fármacos , Anti-Inflamatórios/farmacologiaRESUMO
We propose a universal spin superconducting diode effect (SDE) induced by spin-orbit coupling (SOC) in systems with spin-triplet correlations, where the critical spin supercurrents in opposite directions are unequal. By analysis from both the Ginzburg-Landau theory and energy band analysis, we show that the spin-↑↑ and spin-↓↓ Cooper pairs possess opposite phase gradients and opposite momenta from the SOC, which leads to the spin SDE. Two superconductors with SOC, a p-wave superconductor as a toy model and a practical superconducting nanowire, are numerically studied and they both exhibit spin SDE. In addition, our theory also provides a unified picture for both spin and charge SDEs.
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Cathelicidins are important antimicrobial peptides in various vertebrate species where they are crucial parts of the innate immune system. The current understanding of amphibian cathelicidins is limited, particularly with regard to their immunomodulatory effects. To address this knowledge gap, we produced the cDNA sequence of the cathelicidin gene from a skin transcriptome of the Chinese spiny frog Quasipaa spinosa. The amino acid sequence of the Quasipaa spinosa cathelicidin (QS-CATH) was predicted to consist of a signal peptide, a cathelin domain, and a mature peptide. Comparative analysis of the QS-CATH amino acid sequence with that of other amphibian cathelicidins revealed high variability in the functional mature peptide among amphibians, whereas the cathelin domain was conserved. The QS-CATH gene was expressed in several tissues, with the highest level of expression in the spleen. Upregulation of QS-CATH after Aeromonas hydrophila infection occurred in the kidney, gut, spleen, skin, and liver. Chemically synthesized QS-CATH exhibited pronounced antibacterial activity against Shigella flexneri, Staphylococcus warneri, Escherichia coli, Salmonella enterica, and Listeria monocytogenes. Furthermore, QS-CATH disrupted the cell membrane integrity of S. flexneri, as evidenced by a lactate dehydrogenase release assay, and it hydrolyzed the genomic DNA of S. flexneri. Additionally, QS-CATH elicited chemotaxis and modulated the expression of inflammatory cytokine genes in RAW264.7 mouse leukemic monocyte/macrophage cells. These findings confirm the antimicrobial effects of amphibian cathelicidin and its ability to influence immune cell function. This will expedite the potential utilization of amphibian antimicrobial peptides as therapeutic agents.
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Anuros , Catelicidinas , Animais , Camundongos , Sequência de Aminoácidos , Fatores Imunológicos/farmacologia , Aeromonas hydrophila , Proteínas de Anfíbios/farmacologia , Proteínas de Anfíbios/genética , Proteínas de Anfíbios/isolamento & purificação , Anti-Infecciosos/farmacologia , Antibacterianos/farmacologia , Peptídeos Catiônicos Antimicrobianos/farmacologia , Peptídeos Catiônicos Antimicrobianos/isolamento & purificação , Células RAW 264.7 , Agentes de Imunomodulação/farmacologia , Agentes de Imunomodulação/isolamento & purificação , Pele/efeitos dos fármacos , Pele/metabolismo , Pele/imunologia , População do Leste AsiáticoRESUMO
With the advancement of computer-based testing, log file data has drawn considerable attention from researchers. Although emerging studies have begun to explore log file data, there is a gap in the exploitation of log file data for capturing and understanding participants' cognitive processes. The debate on how to maximize insights from log file data has not yet reached a consensus. Therefore, we present this protocol for a scoping review that aims to characterize the application of log file data in current publications, including the data pre-processing techniques, analytical methodologies, and theoretical frameworks used by researchers. This review will also aim to illuminate how log file data can enhance psychological and educational assessments. Our findings will highlight the opportunities and challenges presented by log file data as an emerging and essential source of evidence for future advancements in psychological and educational assessment.
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Avaliação Educacional , Humanos , Avaliação Educacional/métodos , Testes PsicológicosRESUMO
Linker installation is a potent strategy for integrating specific properties and functionalities into metal-organic frameworks (MOFs). This method enhances the structural diversity of frameworks and enables the precise construction of robust structures, complementing the conventional postsynthetic modification approaches, by fully leveraging open metal sites and active organic linkers at targeting locations. Herein, we demonstrated an insertion of a d-camphorate linker into a flexible Zr-based MOF, PCN-700, through linker installation. The resultant homochiral MOF not only exhibits remarkable stability but also functions as a highly efficient luminescent material for enantioselective sensing. Competitive absorption and energy/electron transfer processes contribute to the sensing performance, while the difference in binding affinities dominates the enantioselectivity. This work presents a straightforward route to crafting stable homochiral MOFs for enantioselective sensing.
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The efficient and ecofriendly removal of pharmaceutical antibiotics and heavy metal Cr(VI) from water sources is a crucial challenge in current environmental management. Photocatalysis presents a viable environmentally friendly solution for eliminating organic contaminants and heavy-metal ions. In this study, a novel S-scheme CuInS2/ZnIn2S4 (CIS/ZIS) heterojunction was developed using a one-pot solvothermal method. The optimized CIS/ZIS heterojunction exhibited considerably improved photocatalytic activity for the removal of antibiotics and Cr(VI), achieving over 90% removal for both tetracycline hydrochloride (TC) (20 mg/L) and Cr(VI) (20 mg/L) under visible light irradiation. The study also delved into the effect of coexisting inorganic anions and assessed the cyclic stability of the composite photocatalysts. This enhancement mechanism can be delineated into three key elements. First, the incorporation of the narrow-gap semiconductor CuInS2 effectively augmented the photoabsorption capacity. Second, the inclusion of ZnIn2S4 caused an increase in surface active sites. Most importantly, the internal electric field at the interface between CuInS2 and ZnIn2S4 expedited the separation of photogenerated carriers. Furthermore, the results revealed that superoxide radical and photogenerated holes are the primary active substance responsible for TC removal, while photogenerated electrons play a central role in the photoreduction of Cr(VI). To gain insights into the transport pathways of photogenerated carriers, we conducted experiments with nitrotetrazolium blue chloride (NBT) and photodeposited gold. This study offers an innovative approach to enhancing the photocatalytic performance of ternary In-based materials by constructing S-scheme heterojunctions.
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Antibacterianos , Cromo , Eletricidade , ElétronsRESUMO
BACKGROUND: Antibiotics biosynthesis is usually regulated by the cluster-situated regulatory gene(s) (CSRG(s)), which directly regulate the genes within the corresponding biosynthetic gene cluster (BGC). Previously, we have demonstrated that LmbU functions as a cluster-situated regulator (CSR) of lincomycin. And it has been found that LmbU regulates twenty non-lmb genes through comparative transcriptomic analysis. However, the regulatory mode of CSRs' targets outside the BGC remains unknown. RESULTS: We screened the targets of LmbU in the whole genome of Streptomyces lincolnensis and found fourteen candidate targets, among which, eight targets can bind to LmbU by electrophoretic mobility shift assays (EMSA). Reporter assays in vivo revealed that LmbU repressed the transcription of SLINC_0469 and SLINC_1037 while activating the transcription of SLINC_8097. In addition, disruptions of SLINC_0469, SLINC_1037, and SLINC_8097 promoted the production of lincomycin, and qRT-PCR showed that SLINC_0469, SLINC_1037, and SLINC_8097 inhibited transcription of the lmb genes, indicating that all the three regulators can negatively regulate lincomycin biosynthesis. CONCLUSIONS: LmbU can directly regulate genes outside the lmb cluster, and these genes can affect both lincomycin biosynthesis and the transcription of lmb genes. Our results first erected the cascade regulatory circuit of LmbU and regulators outside lmb cluster, which provides the theoretical basis for the functional research of LmbU family proteins.
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Proteínas de Bactérias , Streptomyces , Proteínas de Bactérias/genética , Proteínas de Bactérias/metabolismo , Lincomicina , Streptomyces/genética , Streptomyces/metabolismo , Transcriptoma , Regulação Bacteriana da Expressão Gênica , Antibacterianos/farmacologia , Antibacterianos/metabolismoRESUMO
Radical cascade cyclization via the cracking of alkenyl C-H has emerged as an attractive and remarkable tool for the rapid construction of ring frameworks with endocyclic double bonds. We developed a cascade reaction of 3-aza-1,5-enynes with sulfur dioxide and cycloketone oxime esters to access cyanoalkylsulfonylated 1,2-dihydropyridines, which can be easily converted to pyridine derivatives. This protocol involves radical addition to the C≡C bond and 6-endo cyclization and features high regioselectivity and a broad substrate scope.
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Background: Lung cancer is the second most common form of malignant tumor and has the highest mortality rate worldwide. Among its subtypes, lung adenocarcinoma is the most prevalent. Leptomeningeal metastasis (LM) is rare and is characterized by a dismal prognosis, with overall survival periods typically spanning 4 to 6 weeks without treatment. However, in specific cases, survival can be extended to 4 to 6 months with appropriate therapy. The recent approval of third-generation tyrosine kinase inhibitors (TKIs), such as osimertinib, aumolertinib, and furmonertinib, has introduced promising treatment options for individuals with non-small cell lung cancer (NSCLC) who develop LM after developing resistance to first- and second-generation TKIs. These third-generation TKIs exhibit an enhanced ability to penetrate the blood-brain barrier (BBB), opening up new avenues for managing this challenging condition. Case summary: We report the case of a 48-year-old Chinese man diagnosed with advanced NSCLC harboring an epidermal growth factor receptor (EGFR) mutation. Following a pulmonary lobectomy and postoperative adjuvant therapy with gefitinib, the patient was diagnosed with LM, which was confirmed by his neurologic symptoms, cerebrospinal fluid cytologic analysis, and cranial enhancement magnetic resonance imaging. Subsequently, he received oral treatment in the form of 160 mg of furmonertinib daily. After 5 days of furmonertinib therapy, the patient recovered from lethargy, with an obvious improvement in cognitive function. Follow-up visits revealed a 6-month survival period following the LM diagnosis. Patients with NSCLC and LM typically present with severe symptoms, and the efficacy of systemic treatment, intrathecal chemotherapy, and radiotherapy remains unsatisfactory. We hope that this specific case provide valuable insights into the management of patients with EGFR mutation-associated NSCLC with LM. Conclusion: Furmonertinib, a third-generation EGFR TKI with notable BBB penetration, shows promise in LM control and the rapid alleviation of intracranial symptoms. Further investigations into appropriate dosage and toxicity management are imperative.
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Regulators belonging to the DeoR family are widely distributed among the bacteria. Few studies have reported that DeoR family proteins regulate secondary metabolism of Streptomyces. This study explored the function of DeoR (SLINC_8027) in Streptomyces lincolnensis. Deletion of deoR in NRRL 2936 led to an increase in cell growth. The lincomycin production of the deoR deleted strain ΔdeoR was 3.4-fold higher than that of the wild strain. This trait can be recovered to a certain extent in the deoR complemented strain ΔdeoR::pdeoR. According to qRT-PCR analysis, DeoR inhibited the transcription of all detectable genes in the lincomycin biosynthesis cluster and repressed the expression of glnR, bldD, and SLCG_Lrp, which encode regulators outside the cluster. DeoR also inhibited the transcription of itself, as revealed by the XylE reporter. Furthermore, we demonstrated that DeoR bound directly to the promoter region of deoR, lmbA, lmbC-D, lmbJ-K, lmrA, lmrC, glnR, and SLCG_Lrp, by recognizing the 5'-CGATCR-3' motif. This study found that versatile regulatory factor DeoR negatively regulates lincomycin biosynthesis and cellular growth in S. lincolnensis, which expanded the regulatory network of lincomycin biosynthesis.
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Lincomicina , Streptomyces , Lincomicina/metabolismo , Proteínas de Bactérias/genética , Proteínas de Bactérias/metabolismo , Streptomyces/genética , Streptomyces/metabolismo , Metabolismo Secundário , Regulação Bacteriana da Expressão GênicaRESUMO
Sodium-ion batteries (SIBs) are expected to replace partial reliance on lithium-ion batteries (LIBs) in the field of large-scale energy storage as well as low-speed electric vehicles due to the abundance, wide distribution, and easy availability of sodium metal. Unfortunately, a certain amount of sodium ions are irreversibly trapped in the solid electrolyte interface (SEI) layer during the initial charging process, causing the initial capacity loss (ICL) of the SIBs. A separator capacity-compensation strategy is proposed, where the capacity compensator on the separator oxidizes below the high cut-off voltage of the cathode to provide additional sodium ions. This strategy shows attractive advantages, including adaptability to current production processes, no impairment of cell long-cycle life, controlled pre-sodiation degree, and strategy universality. The separator capacity-compensation strategy is applied in the NaNi1/3 Fe1/3 Mn1/3 O2 (NMFO)||HC full cell and achieve a compensated capacity ratio of 18.2%. In the Na3 V2 (PO4 )3 (NVP)||HC full cell, the initial reversible specific capacity is increased from 61.0 mAh g-1 to 83.1 mAh g-1 . The separator capacity-compensation strategy is proven to be universal and provides a new perspective to enhance the energy density of SIBs.
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The hitting position and velocity control for table tennis robots have been investigated widely in the literature. However, most of the studies conducted do not consider the opponent's hitting behaviors, which may reduce hitting accuracy. This paper proposes a new table tennis robot framework that returns the ball based on the opponent's hitting behaviors. Specifically, we classify the opponent's hitting behaviors into four categories: forehand attacking, forehand rubbing, backhand attacking, and backhand rubbing. A tailor-made mechanical structure that consists of a robot arm and a two-dimensional slide rail is developed such that the robot can reach large workspaces. Additionally, a visual module is incorporated to enable the robot to capture opponent motion sequences. Based on the opponent's hitting behaviors and the predicted ball trajectory, smooth and stable control of the robot's hitting motion can be obtained by applying quintic polynomial trajectory planning. Moreover, a motion control strategy is devised for the robot to return the ball to the desired location. Extensive experimental results are presented to demonstrate the effectiveness of the proposed strategy.
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Lincomycin produced by Streptomyces lincolnensis is a critical antibacterial antibiotic in the clinical. To further understand the regulatory mechanism of lincomycin biosynthesis, we identified an alternative σ factor, σL sl , in Streptomyces lincolnensis NRRL 2936. Deletion of sigLsl resulted in an increase in cell growth but a decrease in lincomycin production. σL sl boosted lincomycin biosynthesis by directly stimulating the transcription of four genes (lmbD, lmbV, lmrC, and lmbU) within the lincomycin biosynthetic lmb gene cluster. Besides, σL sl participated in lincomycin biosynthesis by directly stimulating the transcription of mshC, a gene responsible for MSH synthesis. In conclusion, our findings demonstrated that σL sl plays a direct regulatory role in lincomycin biosynthesis. This study extends the understanding of molecular mechanisms of lincomycin biosynthetic regulation.
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Lincomicina , Fator sigma , Fator sigma/genética , Proteínas de Bactérias/genética , AntibacterianosRESUMO
BACKGROUND: Nursing is a high-risk profession and nurses' exposure to workplace risk factors such as heavy workloads and inadequate staffing is well documented. The COVID-19 pandemic has exacerbated nurses' exposure to workplace risk factors, further deteriorating their mental health. Therefore, it is both timely and important to determine nursing groups in greatest need of mental health interventions and supports. PURPOSE: The purpose of this study is to provide a granular examination of the differences in nurse mental health across nurse demographic and workplace characteristics before and after COVID-19 was declared a pandemic. METHODS: This secondary analysis used survey data from two cross-sectional studies with samples (Time 1 study, 5,512 nurses; Time 2, 4,523) recruited from the nursing membership (â¼48,000) of the British Columbia nurses' union. Data was analyzed at each timepoint using descriptive statistics and ordinal logistic regression. RESULTS: Several demographic and workplace characteristics were found to predict significant differences in the number of positive screenings on measures of poor mental health. Most importantly, in both survey times younger age was a strong predictor of worse mental health, as was full-time employment. Nurse workplace health authority was also a significant predictor of worse mental health. CONCLUSIONS: Structural and psychological strategies must be in place, proactively and preventively, to buffer nurses against workplace challenges that are likely to increase during the COVID-19 crisis.
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COVID-19 , Enfermeiras e Enfermeiros , Humanos , Saúde Mental , COVID-19/epidemiologia , Estudos Transversais , Pandemias , Local de Trabalho , Inquéritos e QuestionáriosRESUMO
RATIONALE: Tau hyperphosphorylation and aggregation is considered as a main pathological mechanism underlying Alzheimer's disease (AD). Rose Bengal (RB) is a synthetic dye used for disease diagnosis, which was reported to inhibit tau toxicity via inhibiting tau aggregation in Drosophila. However, it was unknown if RB could produce anti-AD effects in rodents. OBJECTIVES: The research aimed to investigate if and how RB could prevent ß-amyloid (Aß) oligomers-induced tau hyperphosphorylation in rodents. METHODS AND RESULTS: RB was tested in vitro (0.3-1 µM) and prevented Aß oligomers-induced tau hyperphosphorylation in PC12 cells. Moreover, RB (10-30 mg/kg, i.p.) effectively attenuated cognitive impairments induced by Aß oligomers in mice. Western blotting analysis demonstrated that RB significantly increased the expression of pSer473-Akt, pSer9-glycogen synthase kinase-3ß (GSK3ß) and reduced the expression of cyclin-dependent kinase 5 (CDK5) both in vitro and in vivo. Molecular docking analysis suggested that RB might directly interact with GSK3ß and CDK5 by acting on ATP binding sites. Gene Ontology enrichment analysis indicated that RB might act on protein phosphorylation pathways to inhibit tau hyperphosphorylation. CONCLUSIONS: RB was shown to inhibit tau neurotoxicity at least partially via inhibiting the activity of GSK3ß and CDK5, which is a novel neuroprotective mechanism besides the inhibition of tau aggregation. As tau hyperphosphorylation is an important target for AD therapy, this study also provided support for investigating the drug repurposing of RB as an anti-AD drug candidate.
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Doença de Alzheimer , Peptídeos beta-Amiloides , Ratos , Camundongos , Animais , Peptídeos beta-Amiloides/toxicidade , Peptídeos beta-Amiloides/metabolismo , Quinase 5 Dependente de Ciclina/metabolismo , Proteínas tau/genética , Proteínas Proto-Oncogênicas c-akt/metabolismo , Rosa Bengala/uso terapêutico , Glicogênio Sintase Quinase 3 beta/metabolismo , Simulação de Acoplamento Molecular , Doença de Alzheimer/tratamento farmacológico , Fosforilação , Trifosfato de Adenosina/metabolismo , Trifosfato de Adenosina/uso terapêuticoRESUMO
Nitrilases are promising biocatalysts to produce high-value-added carboxylic acids through hydrolysis of nitriles. However, since the enzymes always show low activity and sometimes with poor reaction specificity toward 2-chloronicotinonitrile (2-CN), very few robust nitrilases have been reported for efficient production of 2-chloronicotinic acid (2-CA) from 2-CN. Herein, a nitrilase from Paraburkholderia graminis (PgNIT) was engineered to improve its catalytic properties. We identified the beneficial residues via computational analysis and constructed the mutant library. The positive mutants were obtained and the activity of the "best" mutant F164G/I130L/N167Y/A55S/Q260C/T133I/R199Q toward 2-CN was increased from 0.14 × 10-3 to 4.22 U/mg. Its reaction specificity was improved with elimination of hydration activity. Molecular docking and molecular dynamics simulation revealed that the conformational flexibility, the nucleophilic attack distance, as well as the interaction forces between the enzyme and substrate were the main reason alternating the catalytic properties of PgNIT. With the best mutant as biocatalyst, 150 g/L 2-CN was completely converted, resulting in 2-CA accumulated to 169.7 g/L. When the substrate concentration was increased to 200 g/L, 203.1 g/L 2-CA was obtained with yield of 85.7%. The results laid the foundation for industrial production of 2-CA with the nitrilase-catalyzed route.
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Aminoidrolases , Burkholderiaceae , Ácidos Nicotínicos , Aminoidrolases/química , Aminoidrolases/genética , Aminoidrolases/metabolismo , Burkholderiaceae/genética , Burkholderiaceae/metabolismo , Simulação de Acoplamento Molecular , Especificidade por Substrato , Ácidos Nicotínicos/biossíntese , Ácidos Nicotínicos/metabolismo , CatáliseRESUMO
Surface enhanced Raman spectroscopy (SERS) is a promising technique for trace determination. More and more attention is focused on hybrid SERS substrates, which coupled with noble metal nanoparticles and carbon-based materials. Herein, expanded graphite (EG) is used to prepare EG-covered support by ultrasonic washing and filtration. Such support is flexible and can be cut into any shape. And the contact angle (θe) for Au nanorods (Au NRs) sol on the EG-covered support was 108.2° and the hydrophobic surface is helpful for Au NRs to construct 'hot spots' during evaporation. The limits of detection (LOD) for crystal violet (CV), thiram, malachite green (MG) and methylene blue (MB) were as low as 1 ppb, 50 ppb, 1 ppb and 1 ppb, respectively. Moreover, a fast and convenient 'paste-sampling' method could be employed for trace contaminants on real samples, because EG-based Au NRs substrate is of flexibility and porosity. Thus, CV residue on shrimp could be determined lower than 1 ppb and thiram residue on grapes could be identified lower than 50 ppb. In addition to high sensitivity, the stability of EG-based Au NRs substrate is also very good. Even after acid/alkali pretreatment (pH = 4â¼10) or 30 min of thermal treatment (T = 20â¼100 °C), the enhancement of the substrate remained stable. What's more, the substrate could be stored as long as 30 days. The highly stable, sensitive, cost-effective and easy-to-produce EG-based Au NRs substrates exhibit a great potential to promote application of SERS for routine analysis.
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Grafite , Nanopartículas Metálicas , Violeta Genciana/análise , Ouro/química , Grafite/química , Limite de Detecção , Nanopartículas Metálicas/química , Análise Espectral Raman/métodos , Tiram/análiseRESUMO
Factor X (FX) deficiency is prevalent in light-chain (AL) amyloidosis but its clinical significance was not investigated deeply. We conducted a retrospective analysis of a consecutive cohort with 207 primary AL amyloidosis patients. FX deficiency was present in 129 patients (62.3%). Those with FX deficiency had higher dFLC (299.6 mg/L vs. 102.3 mg/L, P < 0.001), higher cardiac troponin I (0.05 µg/L vs. 0.02 µg/L, P < 0.001) and N-terminal pro-brain natriuretic peptide (3115 ng/L vs. 392 ng/L, P < 0.001), and more patients with bone marrow plasma cells > 10% (18.0% vs. 4.3%, P = 0.008). The prevalence of FX deficiency increased with the Mayo 2004 stage. FX-deficient patients exhibited inferior overall survival (P < 0.001) and progression-free survival (P < 0.001) than others. Fifty-five patients with FX deficiency received retesting of FX activity after anti-plasma cell therapy. The median variation in FX activity was + 6.8% (range, -24.5% ~ +73.4%). Better improvement of FX activity was observed in patients with complete hematologic response (+18.2% vs. +4.0%, P = 0.036) and at least one organ response (+14.4% vs. +3.4%, P = 0.024). FX deficiency is associated with a heavier disease burden and poorer survival in primary AL amyloidosis. Improvement of FX activity tends to appear in patients with better hematologic and organ responses after chemotherapy.