Your browser doesn't support javascript.
loading
: 20 | 50 | 100
1 - 20 de 8.978
1.
Chem Soc Rev ; 2024 Jun 03.
Article En | MEDLINE | ID: mdl-38826108

Chiral 1,2-bis(2,5-diphenylphospholano)ethane (Ph-BPE) is a class of optimal organic bisphosphine ligands with C2-symmetry. Ph-BPE with its excellent catalytic performance in asymmetric synthesis has attracted much attention of chemists with increasing popularity and is growing into one of the most commonly used organophosphorus ligands, especially in asymmetric catalysis. Over two hundred examples have been reported since 2012. This review presents how Ph-BPE is utilized in asymmetric synthesis and how powerful it is as a chiral ligand or even a catalyst in a wide range of reactions including applications in the total synthesis of bioactive molecules.

2.
Alzheimers Dement ; 2024 Jun 03.
Article En | MEDLINE | ID: mdl-38829680

INTRODUCTION: Individuals referred to as Non-Demented with Alzheimer's Neuropathology (NDAN) exhibit cognitive resilience despite presenting Alzheimer's disease (AD) histopathological signs. Investigating the mechanisms behind this resilience may unveil crucial insights into AD resistance. METHODS: DiI labeling technique was used to analyze dendritic spine morphology in control (CTRL), AD, and NDAN post mortem frontal cortex, particularly focusing on spine types near and far from amyloid beta (Aß) plaques. RESULTS: NDAN subjects displayed a higher spine density in regions distant from Aß plaques versus AD patients. In distal areas from the plaques, NDAN individuals exhibited more immature spines, while AD patients had a prevalence of mature spines. Additionally, our examination of levels of Peptidyl-prolyl cis-trans isomerase NIMA-interacting 1 (Pin1), a protein associated with synaptic plasticity and AD, showed significantly lower expression in AD versus NDAN and CTRL. DISCUSSION: These results suggest that NDAN individuals undergo synaptic remodeling, potentially facilitated by Pin1, serving as a compensatory mechanism to preserve cognitive function despite AD pathology. HIGHLIGHTS: Spine density is reduced near Aß plaques compared to the distal area in CTRL, AD, and NDAN dendrites. NDAN shows higher spine density than AD in areas far from Aß plaques. Far from Aß plaques, NDAN has a higher density of immature spines, AD a higher density of mature spines. AD individuals show significantly lower levels of Pin1 compared to NDAN and CTRL.

4.
Adv Sci (Weinh) ; : e2402380, 2024 Jun 05.
Article En | MEDLINE | ID: mdl-38837633

Simultaneously achieving high-energy-density and high-power-density is a crucial yet challenging objective in the pursuit of commercialized power batteries. In this study, atomic layer deposition (ALD) is employed combined with a coordinated thermal treatment strategy to construct a densely packed, electron-ion dual conductor (EIC) protective coating on the surface of commercial LiNi0.5Co0.2Mn0.3O2 (NCM523) cathode material, further enhanced by gradient Al doping (Al@EIC-NCM523). The ultra-thin EIC effectively suppresses side reactions, thereby enhancing the stability of the cathode-electrolyte interphase (CEI) at high-voltages. The EIC's dual conduction capability provides a potent driving force for Li+ transport at the interface, promoting the formation of rapid ion deintercalation pathways within the Al@EIC-NCM523 bulk phase. Moreover, the strategic gradient doping of Al serves to anchor the atomic spacing of Ni and O within the structure of Al@EIC-NCM523, curbing irreversible phase transitions at high-voltages and preserving the integrity of its layered structure. Remarkably, Al@EIC-NCM523 displays an unprecedented rate capability (114.7 mAh g-1 at 20 C), and a sustained cycling performance (capacity retention of 74.72% after 800 cycles at 10 C) at 4.6 V. These findings demonstrate that the proposed EIC and doping strategy holds a significant promise for developing high-energy-density and high-power-density lithium-ion batteries (LIBs).

5.
Angew Chem Int Ed Engl ; : e202408247, 2024 Jun 05.
Article En | MEDLINE | ID: mdl-38837719

The construction of mechanically responsive materials with reversible shape-shifting, shape-locking, and stretchability holds promise for a wide range of applications in fields such as soft robotics and flexible electronics. Here, we report novel thermoelastic one-dimensional organic-inorganic hybrids (R/S-Hmpy)PbI3 (Hmpy = 2-hydroxymethyl-pyrrolidinium) to show mechanical responses. The single crystals undergo two phase transitions at 310 K and 380 K. When heated to 380 K, they show shape-shifting and expansion along the b-axis by about 13.4%, corresponding to a larger deformation than that of thermally activated shape memory alloys (8.5%), and exhibit a strong actuation force. During cooling process, the stretched crystal shape maintains and a shape-locking phenomenon occurs, which is lifted when the temperature decreasing to 305 K. Meanwhile, due to the introduction of chiral ions, the thermal switching shows a 10-fold second-order nonlinear switching contrast (common values typically below 3-fold). This study presents a thermoelastic actuator based on shape-shifting and -locking of organic-inorganic hybrids for the first time. The dielectric and nonlinear optical switching properties of organic-inorganic hybrids broaden the range of applications of mechanically responsive crystals.

6.
Plant Physiol Biochem ; 212: 108778, 2024 May 31.
Article En | MEDLINE | ID: mdl-38838570

The albino tea cultivar is one of the most important germplasms for key gene mining and high-quality tea producing. In order to elucidate the chlorophyll-deficient mechanism of albino cultivar 'Huangjinya' and its offspring, color difference, photosynthetic pigments and the relevant genes' expression of the tender shoots were comprehensively investigated in this study. Among the tested 16 offspring, 5 exhibited albino phenotype in spring and autumn, 3 showed albino phenotype in spring but normal green in autumn, while the rests were all normal green. The shoot of albino offspring had significantly higher lightness and/or yellowness than that of green ones, and possessed dramatically lower photosynthetic pigments and chlorophyll precursor protochlorophyllide (Pchlide), as well as higher chlorophyll a/chlorophyll b but lower chlorophylls/carotenoids in comparison with green ones. Among the tested genes involved in chlorophyll and carotenoid metabolism pathways, expression of the magnesium protoporphyrin IX monomethyl ester cyclase (CRD), 3,8-divinyl chlorophyllide 8-vinyl reductase (DVR), 5-aminolevulinate dehydratase 1 (HEMB1), 1-deoxy-D-xylulose 5-phosphate synthase 1 (DXS1) and 4-hydroxy-3-methylbut-2-enyl diphosphate reductase (ISPH) was remarkably down-regulated in shoots of the albino offspring. Color difference indices of the offspring were significantly correlated with the levels of photosynthetic pigments and Pchlide, and low level of chlorophylls in shoot of albino offspring was mainly due to conversion obstacle from magnesium protoporphyrin Ⅸ (Mg-Proto IX) to Pchlide which might be attributed to down-regulatory expression of CRD and DVR.

7.
J Acoust Soc Am ; 155(6): 3606-3614, 2024 Jun 01.
Article En | MEDLINE | ID: mdl-38833282

Surface and underwater (S/U) acoustic targets recognition is an important application of passive sonar. It is difficult to distinguish them due to the mixture of underwater target radiation noise and marine environmental noise. In previous studies, although using a single hydrophone was able to identify S/U acoustic targets, there were still a few hydrophones that had poor accuracy. In this paper, S/U acoustic targets recognition using two hydrophones based on Gradient Boosting Decision Tree is proposed, and it is first found out as high as 100% accuracy could be achieved with the implementation of SACLANT 1993 data. The real experimental data are always rare and insufficient. The big training dataset is generated using environmental information by acoustic model named KRAKEN. Simulation and experimental data used in the model are heterogeneous, and the differences between these two kinds of data are assimilated by using vertical linear array feature extraction method. The model realizes the recognition of S/U acoustic targets based on channel information besides source spectrum information. By using the combination of two hydrophones, the surface and underwater targets recognition accuracy reached 1 and 0.9384, while they are only 0.4715 and 0.5620 using a single hydrophone, respectively.

9.
Conserv Biol ; : e14310, 2024 Jun 06.
Article En | MEDLINE | ID: mdl-38842221

Climate change may diminish biodiversity; thus, it is urgent to predict how species' ranges may shift in the future by integrating multiple factors involving more taxa. Bats are particularly sensitive to climate change due to their high surface-to-volume ratio. However, few studies have considered geographic variables associated with roost availability and even fewer have linked the distributions of bats to their thermoregulation and energy regulation traits. We used species distribution models to predict the potential distributions of 12 bat species in China under current and future greenhouse gas emission scenarios (SSP1-2.6 and SSP5-8.5) and examined factors that could affect species' range shifts, including climatic, geographic, habitat, and human activity variables and wing surface-to-mass ratio (S-MR). The results suggest that Ia io, Rhinolophus ferrumequinum, and Rhinolophus rex should be given the highest priority for conservation in future climate conservation strategies. Most species were predicted to move northward, except for I. io and R. rex, which moved southward. Temperature seasonality, distance to forest, and distance to karst or cave were the main environmental factors affecting the potential distributions of bats. We found significant relationships between S-MR and geographic distribution, current potential distribution, and future potential distribution in the 2050s. Our work highlights the importance of analyzing range shifts of species with multifactorial approaches, especially for species traits related to thermoregulation and energy regulation, to provide targeted conservation strategies.


Patrones y correlaciones de los cambios potenciales en la distribución de las especies de murciélago de China en el contexto del cambio climático Resumen El cambio climático puede disminuir la biodiversidad, por lo que es urgente pronosticar cómo puede cambiar en el futuro la distribución de las especies mediante la integración de múltiples factores que involucren a más taxones. Los murciélagos son particularmente sensibles al cambio climático debido a que tienen una gran proporción superficie­volumen. Sin embargo, hay pocos estudios que han considerado las variables asociadas con la disponibilidad de nidos y son todavía menos los que han conectado la distribución de los murciélagos con sus rasgos de termorregulación y regulación de energía. Usamos modelos de distribución de especies para pronosticar la distribución potencial de doce especies de murciélago en China bajo escenarios actuales y futuros de emisión de gases de efecto invernadero (SSP1­2.6 y SSP5­8.5) y analizamos los factores que podrían afectar el cambio en la distribución de las especies, incluyendo las variables climáticas, geográficas, de hábitat y de actividad humana y la proporción entre la superficie del ala y la masa (P S­M). Los resultados sugieren que Ia io, Rhinolophus ferrumequinum y R. rex deberían ser la mayor prioridad de conservación para las estrategias de conservación climáticas en el futuro. Pronosticamos que la mayoría de las especies se desplazarían al norte, a excepción de I. io y R. rex, que se desplazarían hacia el sur. Los principales factores que afectaron la distribución potencial de los murciélagos fueron la estacionalidad de la temperatura, la distancia al bosque y la distancia a la cueva o al karst. Encontramos una relación significativa entre la P S­M y la distribución geográfica, la distribución potencial actual y la distribución potencial para la década de 2050. Nuestra investigación destaca la importancia del análisis de los cambios de distribución de las especies con enfoques multifactoriales, especialmente para los rasgos de especie relacionados con la termorregulación y la regulación de energía, para proporcionar estrategias de conservación focalizadas.

11.
Cardiovasc Diabetol ; 23(1): 187, 2024 May 31.
Article En | MEDLINE | ID: mdl-38822373

BACKGROUND: Ischemia and no obstructive coronary artery disease (INOCA) is increasingly recognized and associated with poor outcomes. The triglyceride-glucose (TyG) index is a reliable alternative measure of insulin resistance significantly linked to cardiovascular disease and adverse prognosis. We investigated the association between the TyG index and myocardial ischemia and the prognosis in INOCA patients. METHODS: INOCA patients who underwent both coronary angiography and myocardial perfusion imaging (MPI) were included consecutively. All participants were divided into three groups according to TyG tertiles (T1, T2, and T3). Abnormal MPI for myocardial ischemia in individual coronary territories was defined as summed stress score (SSS) ≥ 4 and summed difference score (SDS) ≥ 2. SSS refers to the sum of all defects in the stress images, and SDS is the difference of the sum of all defects between the rest images and stress images. All patients were followed up for major adverse cardiac events (MACE). RESULTS: Among 332 INOCA patients, 113 (34.0%) had abnormal MPI. Patients with higher TyG index had a higher rate of abnormal MPI (25.5% vs. 32.4% vs. 44.1%; p = 0.012). Multivariate logistic analysis showed that a high TyG index was significantly correlated with abnormal MPI in INOCA patients (OR, 1.901; 95% CI, 1.045-3.458; P = 0.035). During the median 35 months of follow-up, 83 (25%) MACE were recorded, and a higher incidence of MACE was observed in the T3 group (T3 vs. T2 vs. T1: 36.9% vs. 21.6% vs. 16.4%, respectively; p = 0.001). In multivariate Cox regression analysis, the T3 group was significantly associated with the risk of MACE compared to the T1 group (HR, 2.338; 95% CI 1.253-4.364, P = 0.008). CONCLUSION: This study indicates for the first time that the TyG index is significantly associated with myocardial ischemia and poor prognosis among INOCA patients.


Biomarkers , Blood Glucose , Coronary Angiography , Myocardial Ischemia , Myocardial Perfusion Imaging , Predictive Value of Tests , Triglycerides , Humans , Male , Female , Middle Aged , Aged , Triglycerides/blood , Prognosis , Myocardial Ischemia/blood , Myocardial Ischemia/diagnosis , Myocardial Ischemia/mortality , Myocardial Ischemia/epidemiology , Biomarkers/blood , Blood Glucose/metabolism , Risk Factors , Risk Assessment , Retrospective Studies , Time Factors , Coronary Artery Disease/blood , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/diagnosis , Coronary Artery Disease/mortality , Insulin Resistance
12.
Se Pu ; 42(6): 572-580, 2024 Jun.
Article Zh | MEDLINE | ID: mdl-38845518

Perfluorooctanoic acid (PFOA) is a persistent contaminant with detrimental effects on the natural environment. This persistence leads to potential enrichment and osmotic transfer, which can affect normal circulation in the environment. PFOA poses significant threats to both the natural environment and human health. Therefore, the development of cost-effective, highly efficient, and environment-friendly PFOA adsorbents is a crucial endeavor. This paper presents the catalyst-free one-pot synthesis of fluorinated nitrogen-rich porous organic polymers (POP-3F) via a Schiff-base condensation reaction. The reaction between the nitrogen-rich compound 1,4-bis(2,4-diamino-1,3,5-triazine)benzene and p-trifluoromethylbenzaldehyde yielded POP-3F. The introduction of fluorine atoms into the nitrogen-rich porous organic polymer enhanced its hydrophobicity, thereby facilitating favorable fluoro-fluorine interactions with PFOA and, thus, improving the efficacy of the adsorbent. Scanning electron microscopy (SEM), Fourier transform infrared (FT-IR) spectroscopy, X-ray diffraction (XRD), solid-state nuclear magnetic resonance (ssNMR) spectroscopy, X-ray photoelectron spectroscopy (XPS), nitrogen adsorption-desorption analysis, and thermogravimetric analysis (TGA) were used to confirm the successful synthesis and characterization of POP-3F. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) was conducted in negative electrospray ionization (ESI) mode coupled with multi-reaction monitoring mode (MRM). The instrument was equipped with an Atlantis T3 column (100 mm×2.1 mm, 3 µm), and analysis was conducted using an external standard method. The influences of various factors on PFOA adsorption by POP-3F, including pH, salt concentration, and humic acid presence, were investigated. The highest PFOA removal rate (98.6%) was achieved at a pH of 2, indicating the applicability of POP-3F for the effective removal of PFOA from acidic industrial wastewater. The removal rate of PFOA was unaffected by increases in NaCl concentration. This phenomenon can be attributed to electrostatic interactions between the protonated secondary amines in POP-3F and deprotonated PFOA. Upon the addition of NaCl, a double electric layer is formed on the POP-3F surface, with Cl- ions in the outer layer and Na+ ions in the inner layer, which weakened these interactions. Humic acid is competitively adsorbed with PFOA. However, POP-3F demonstrated good removal rates even in the presence of high humic acid concentrations in water. Adsorption isotherm and kinetics experiments were conducted at the optimal pH to explore the relevant adsorption mechanism. The results showed a rapid initial adsorption rate, with 95.4% PFOA removal within 5 min. Optimal adsorption equilibrium was achieved within 6 h, and the removal rate decreased by only 0.3% after 24 h. This finding indicates that POP-3F exhibits sustained efficacy for PFOA removal. Langmuir fitting analysis revealed a theoretical maximum adsorption capacity of 191 mg/g for POP-3F; this value surpasses those of activated carbon materials and most other adsorbents, highlighting the superior PFOA-adsorption performance of POP-3F. Additionally, matrix effects minimally affected the removal of PFOA by POP-3F, with only a slight reduction (0.1%) observed in simulated natural water. The recyclability of POP-3F was assessed over five adsorption-desorption cycles. The removal efficenecy exhibited a minor decrease of only 0.67% after five cycles. These results demonstrate the recyclability of the proposed adsorbent, which translates into cost reduction through reusability. This characteristic renders POP-3F a promising candidate for the economical and efficient removal of PFOA from wastewater in practical applications.

13.
Sci Adv ; 10(23): eadk9996, 2024 Jun 07.
Article En | MEDLINE | ID: mdl-38838152

Immunotoxicity remains a major hindrance to chemotherapy in cancer therapy. Nanocarriers may alleviate the immunotoxicity, but the optimal design remains unclear. Here, we created two variants of maytansine (DM1)-loaded synthetic high-density lipoproteins (D-sHDL) with either physically entrapped (ED-sHDL) or chemically conjugated (CD-sHDL) DM1. We found that CD-sHDL showed less accumulation in the tumor draining lymph nodes (DLNs) and femur, resulting in a lower toxicity against myeloid cells than ED-sHDL via avoiding scavenger receptor class B type 1 (SR-B1)-mediated DM1 transportation into the granulocyte-monocyte progenitors and dendritic cells. Therefore, higher densities of lymphocytes in the tumors, DLNs, and blood were recorded in mice receiving CD-sHDL, leading to a better efficacy and immune memory of CD-sHDL against colon cancer. Furthermore, liposomes with conjugated DM1 (CD-Lipo) showed lower immunotoxicity than those with entrapped drug (ED-Lipo) through the same mechanism after apolipoprotein opsonization. Our findings highlight the critical role of drug loading patterns in dictating the biological fate and activity of nanomedicine.


Nanoparticles , Animals , Nanoparticles/chemistry , Mice , Cell Line, Tumor , Humans , Scavenger Receptors, Class B/metabolism , Antineoplastic Agents/pharmacology , Antineoplastic Agents/chemistry , Lipoproteins, HDL/metabolism , Drug Carriers/chemistry , Colonic Neoplasms/drug therapy , Colonic Neoplasms/immunology , Colonic Neoplasms/metabolism , Colonic Neoplasms/pathology , Liposomes/chemistry , Lipids/chemistry
14.
ACS Omega ; 9(21): 23053-23059, 2024 May 28.
Article En | MEDLINE | ID: mdl-38826519

In this paper, we present a study of the thermal transport of epitaxial bilayer graphene microbridges. The thermal conductance of three graphene microbridges with different lengths was measured at different temperatures using Johnson noise thermometry. We find that with the decrease of the temperature, the thermal transport in the graphene microbridges switches from electron-phonon coupling to electron diffusion, and the switching temperature is dependent on the length of the microbridge, which is in good agreement with the simulation based on a distributed hot-spot model. Moreover, the electron-phonon thermal conductance has a temperature power law of T3 as predicted for pristine graphene and the electron-phonon coupling coefficient σep is found to be approximately 0.18 W/(m2 K4), corresponding to a deformation potential D of 55 eV. In addition, the electron diffusion in the graphene microbridges adheres to the Wiedemann-Franz law, requiring no corrections to the Lorentz number.

15.
Sci Rep ; 14(1): 12716, 2024 06 03.
Article En | MEDLINE | ID: mdl-38830933

To explore the molecular pathogenesis of pulmonary arterial hypertension (PAH) and identify potential therapeutic targets, we performed transcriptome sequencing of lung tissue from mice with hypoxia-induced pulmonary hypertension. Our Gene Ontology analysis revealed that "extracellular matrix organization" ranked high in the biological process category, and matrix metallopeptidases (MMPs) and other proteases also played important roles in it. Moreover, compared with those in the normoxia group, we confirmed that MMPs expression was upregulated in the hypoxia group, while the hub gene Timp1 was downregulated. Crocin, a natural MMP inhibitor, was found to reduce inflammation, decrease MMPs levels, increase Timp1 expression levels, and attenuate hypoxia-induced pulmonary hypertension in mice. In addition, analysis of the cell distribution of MMPs and Timp1 in the human lung cell atlas using single-cell RNAseq datasets revealed that MMPs and Timp1 are mainly expressed in a population of fibroblasts. Moreover, in vitro experiments revealed that crocin significantly inhibited myofibroblast proliferation, migration, and extracellular matrix deposition. Furthermore, we demonstrated that crocin inhibited TGF-ß1-induced fibroblast activation and regulated the pulmonary arterial fibroblast MMP2/TIMP1 balance by inhibiting the TGF-ß1/Smad3 signaling pathway. In summary, our results indicate that crocin attenuates hypoxia-induced pulmonary hypertension in mice by inhibiting TGF-ß1-induced myofibroblast activation.


Carotenoids , Hypertension, Pulmonary , Hypoxia , Matrix Metalloproteinase 2 , Tissue Inhibitor of Metalloproteinase-1 , Animals , Tissue Inhibitor of Metalloproteinase-1/metabolism , Tissue Inhibitor of Metalloproteinase-1/genetics , Mice , Hypoxia/metabolism , Hypoxia/complications , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/metabolism , Carotenoids/pharmacology , Humans , Matrix Metalloproteinase 2/metabolism , Matrix Metalloproteinase 2/genetics , Male , Signal Transduction/drug effects , Transforming Growth Factor beta1/metabolism , Disease Models, Animal , Cell Proliferation/drug effects , Mice, Inbred C57BL , Smad3 Protein/metabolism , Cell Movement/drug effects , Lung/pathology , Lung/metabolism , Lung/drug effects
16.
J Clin Lab Anal ; : e25074, 2024 Jun 07.
Article En | MEDLINE | ID: mdl-38847175

OBJECTIVE: To establish the reference range of serum concentration of vitamin A (VA) and vitamin E (VE) in Southern Sichuan area of China. METHODS: From August 1, 2021, to May 31, 2023, 9482 blood tablets were received for the screening of VA and VE. The information was divided into four different age groups: ≤1 year old, 1< to ≤6 years, 6< to ≤17 years, and 17< to ≤59 years. In each age group, the four seasons were further subdivided into spring, summer, autumn, and winter, as well as male and female genders. The serum concentration of VA and VE was detected by liquid chromatography-tandem mass spectrometry (HPLC-MS), and the reference range was established for verification. RESULTS: The concentration of VA and VE in 9482 cases showed skewed distribution. When comparing between different age groups, the serum concentration of VA and VE was statistically significant (p < 0.05). While comparing different seasons, the serum VA levels in different seasons were significantly different (p < 0.05) except in summer and autumn. There was statistical significance in VE level in different seasons (p < 0.05). And while comparing different genders, there was no statistical significance in VA concentration levels (p > 0.05). The VE concentration levels were statistically significant (p < 0.05). The established reference range was established and verified, and the results were in accordance with the standard. CONCLUSION: The reference range of VA and VE should be set according to different ages, different seasons, and different genders.

17.
Liver Int ; 2024 Jun 07.
Article En | MEDLINE | ID: mdl-38847512

BACKGROUND AND AIMS: Few studies have focused on the outcomes of Wilson's disease (WD) diagnosed before age of 5 years. This study aimed to summarize the clinical features of early diagnosed WD and analyse treatment outcomes and the risk factors associated with treatment failure. METHODS: A total of 139 children confirmed with WD before 5 years were enrolled in this study. Only patients with follow-up over 1 year were analysed with Kaplan-Meier survival analysis. The composite outcomes included death, progression to liver failure or acute hepatitis, development of renal or neurological symptoms and persistent elevation of alanine aminotransferase (ALT). The treatment failure was defined as occurrence of at least one of above outcomes. RESULTS: Among 139 WD patients at diagnosis, two (1.4%) WD patients presented with symptomatic liver disease, whereas 137 (98.6%) were phenotypically asymptomatic, including 135 with elevated ALT and 2 with normal liver function. Median serum ceruloplasmin (Cp) was 3.1 mg/dL, and urinary copper excretion was 87.4 µg/24-h. There were 71 variants identified in the the copper-transporting ATPase beta gene, and 29 were loss of function (LOF). 51 patients with LOF variant were younger at diagnosis and had lower Cp than 88 patients without LOF. Among 93 patients with over 1 year of follow-up, 19 (20.4%) received zinc monotherapy, and 74 (79.6%) received a zinc/D-penicillamine combination therapy. 14 (15.1%) patients underwent treatment failure, and its occurrence was associated with poor compliance (p < .01). CONCLUSIONS: Cp is a reliable biomarker for early diagnosis, and zinc monotherapy is an effective treatment for WD during early childhood. Good treatment compliance is critical to achieve a favourable outcome.

18.
Prep Biochem Biotechnol ; : 1-9, 2024 Jun 07.
Article En | MEDLINE | ID: mdl-38847777

Background: Recombinant myofibril-bound serine proteinase (rMBSP) was successfully expressed in Pichia pastoris GS115 in our laboratory. However, low production of rMBSP in shake flask constraints further exploration of properties.Methods: A 5-L high cell density fermentation was performed and the fermentation medium was optimized. Response surface methodology (RSM) was used to optimize the culture condition through modeling three selected parameter.Results: Under the optimized culture medium (LBSM, 1% yeast powder and 1% peptone) and culture conditions (induction pH 5.5, temperature 29 °C, time 40 h), the yield of rMBSP was 420 mg/L in a 5-L fermenter, which was a 6-fold increase over thar, expressed in flask cultivation. The desired enzyme was purified by two-step, which yielded a 33.7% recovery of a product that had over 85% purity. The activity of purified rMBSP was significantly inhibited by Ca2+, Mg2+, SDS, guanidine hydrochloeide, acetone, isopropanol, chloroform, n-hexane and n-heptane. Enzymatic analysis revealed a Km of 2.89 ± 0.09 µM and a Vmax of 14.20 ± 0.12 nM•min-1 for rMBSP. LC-MS/MS analysis demonstrated the specific cleavage of bovine serum albumin by rMPSP.Conclusion: These findings suggest that rMPSP has potential as a valuable enzyme for protein science research.

19.
Front Med (Lausanne) ; 11: 1352206, 2024.
Article En | MEDLINE | ID: mdl-38725466

Background: Kimura disease is characterized by inflammation, with its underlying causes remaining uncertain. There is a lack of comprehensive and systematic research on the pathology of this condition in pediatric patients. Our objective is to study the clinical and pathological attributes of Kimura disease in pediatric patients and investigate the potential diagnostic significance of immunoglobulin E (IgE) in this context. Methods: Clinical and laboratory information, pathological characteristics, and follow-up data were correlated to examine the distinctive features. Immunohistochemistry, acid-fast staining, and molecular assay were used to identify the presence of IgE and pathogens. Results: We conducted an analysis of five cases of Kimura disease in pediatric patients at our hospital. The patients' ages ranged from 5 years and 7 months to 14 years and 2 months, with 4 (80%) being male. The most common site was the head and neck region, particularly the postauricular subcutaneous area. Eosinophilia was observed in four patients (80%), and two patients (40%) had elevated serum immunoglobulin E (IgE) levels. Histopathological changes included eosinophilic infiltrates, follicular hyperplasia, and the proliferation of postcapillary venules. Immunohistochemical results supported the reactive nature of the lymphoid process and IgE deposition in the follicle, while no specific pathogens were discovered by special staining. All patients underwent surgical excision, and none experienced recurrence in their original location. Conclusion: Children with Kimura disease show distinct eosinophilic and IgE alterations in both laboratory findings and pathological features. The application of immunohistochemical staining of IgE could serve as a promising marker for diagnosing Kimura disease.

20.
ACS Biomater Sci Eng ; 2024 May 09.
Article En | MEDLINE | ID: mdl-38722544

Cadmium poses a severe health risk, impacting various bodily systems. Monitoring human exposure is vital. Urine and blood cadmium serve as critical biomarkers. However, current urine and blood cadmium detection methods are expensive and complex. Being cost-effective, user-friendly, and efficient, visual biosensing offers a promising complement to existing techniques. Therefore, we constructed a cadmium whole-cell biosensor using CadR10 and deoxyviolacein pigment in this study. We assessed the sensor for time-dose response, specific response to cadmium, sensitivity response to cadmium, and stability response to cadmium. The results showed that (1) the sensor had a preferred signal-to-noise ratio when the incubation time was 4 h; (2) the sensor showed excellent specificity for cadmium compared to the group 12 metals and lead; (3) the sensor was responsive to cadmium down to 1.53 nM under experimental conditions and had good linearity over a wide range from 1.53 nM to 100 µM with good linearity (R2 = 0.979); and (4) the sensor had good stability. Based on the excellent results of the performance tests, we developed a cost-effective, high-throughput method for detecting urinary and blood cadmium. Specifically, this was realized by adding the blood or urine samples into the culture system in a particular proportion. Then, the whole-cell biosensor was subjected to culture, n-butanol extraction, and microplate reading. The results showed that (1) at 20% urine addition ratio, the sensor had an excellent curvilinear relationship (R2 = 0.986) in the range of 3.05 nM to 100 µM, and the detection limit could reach 3.05 nM. (2) At a 10% blood addition ratio, the sensor had an excellent nonlinear relationship (R2 = 0.978) in the range of 0.097-50 µM, and the detection limit reached 0.195 µM. Overall, we developed a sensitive and wide-range method based on a whole-cell biosensor for the detection of cadmium in blood and urine, which has the advantages of being cost-effective, ease of operation, fast response, and low dependence on instrumentation and has the potential to be applied in the monitoring of cadmium exposure in humans as a complementary to the mainstream detection techniques.

...