Gene variants and clinical characteristics of children with sitosterolemia.

OBJECTIVE: To enhance the detection, management and monitoring of Chinese children afflicted with sitosterolemia by examining the physical characteristics and genetic makeup of pediatric patients. METHODS: In this group, 26 children were diagnosed with sitosterolemia, 24 of whom underwent genetic analysis. Patient family medical history, physical symptoms, tests for liver function, lipid levels, standard blood tests, phytosterol levels, cardiac/carotid artery ultrasounds, fundus examinations, and treatment were collected. RESULTS: The majority (19, 73.1%) of the 26 patients exhibited xanthomas as the most prevalent manifestation. The second most common symptoms were joint pain (7, 26.9%) and stunted growth (4, 15.4%). Among the 24 (92.3%) patients whose genetics were analyzed, 16 (66.7%) harbored ABCG5 variants (type 2 sitosterolemia), and nearly one-third (8, 33.3%) harbored ABCG8 variants (type 1 sitosterolemia). Additionally, the most common pathogenic ABCG5 variant was c.1166G > A (p.Arg389His), which was found in 10 patients (66.7%). Further analysis did not indicate any significant differences in pathological traits among those carrying ABCG5 and ABCG8 variations (P > 0.05). Interestingly, there was a greater abundance of nonsense variations in ABCG5 than in ABCG8 (P = 0.09), and a greater frequency of splicing variations in ABCG8 than ABCG5 (P = 0.01). Following a change in diet or a combination of ezetimibe, the levels of cholesterol and low-density lipoprotein were markedly decreased compared to the levels reported before treatment. CONCLUSION: Sitosterolemia should be considered for individuals presenting with xanthomas and increased cholesterol levels. Phytosterol testing and genetic analysis are important for early detection. Managing one's diet and taking ezetimibe can well control blood lipids.

Recent advances in super-resolution optical imaging based on aggregation-induced emission.

Super-resolution imaging has rapidly emerged as an optical microscopy technique, offering advantages of high optical resolution over the past two decades; achieving improved imaging resolution requires significant efforts in developing super-resolution imaging agents characterized by high brightness, high contrast and high sensitivity to fluorescence switching. Apart from technical requirements in optical systems and algorithms, super-resolution imaging relies on fluorescent dyes with special photophysical or photochemical properties. The concept of aggregation-induced emission (AIE) was proposed in 2001, coinciding with unprecedented advancements and innovations in super-resolution imaging technology. AIE probes offer many advantages, including high brightness in the aggregated state, low background signal, a larger Stokes shift, ultra-high photostability, and excellent biocompatibility, making them highly promising for applications in super-resolution imaging. In this review, we summarize the progress in implementation methods and provide insights into the mechanism of AIE-based super-resolution imaging, including fluorescence switching resulting from photochemically-converted aggregation-induced emission, electrostatically controlled aggregation-induced emission and specific binding-regulated aggregation-induced emission. Particularly, the aggregation-induced emission principle has been proposed to achieve spontaneous fluorescence switching, expanding the selection and application scenarios of super-resolution imaging probes. By combining the aggregation-induced emission principle and specific molecular design, we offer some comprehensive insights to facilitate the applications of AIEgens (AIE-active molecules) in super-resolution imaging.

Towards Optical Information Recording: A Robust Visible-Light-Driven Molecular Photoswitch with the Ring-Closure Reaction Yield Exceeding 96.3 .

Diarylethene molecular photoswitches hold great fascination as optical information materials due to their unique bistability and exceptional reversible photoswitching properties. Conventional diarylethenes, however, rely on UV light for ring-closure reactions, typically with modest yields. For practical application, diarylethenes driven by visible lights are preferred but achieving high ring-closure reaction yield remains a significant challenge. Herein, we synthesized a novel all-visible-light-driven photoswitch, TPAP-DTE, by facilely endcapping the dithienylethene (DTE) core with triphenylamine phenyl (TPAP) groups. Owing to the electron-donating conjugation effect of TPAP, the open-form TPAP-DTE responds strongly to short-wavelength visible lights with considerable photocyclization quantum yields and molar absorption coefficient. Upon 405 nm visible-light irradiation, TPAP-DTE achieves a ring-closure reaction yield exceeding 96.3 % (confirmed by both nuclear magnetic resonance spectroscopy and high-performance liquid chromatography). Its ring-opening reaction yield is 100 % upon irradiation with long-wavelength visible light. TPAP-DTE could be regarded as a bidirectional "quasi"-quantitative conversion molecular switch. Furthermore, TPAP-DTE exhibits robust fatigue resistance over 100 full photoswitching cycles and great anti-aging property under 85 °C and 85 % humidity for at least 1000 h. Consequently, its rewritable QR-code, multilevel data storage, and anti-counterfeiting/encryption applications are successfully demonstrated exclusively using visible lights, positioning TPAP-DTE as a highly promising medium for information recording.

Dual-crosslinked bioadhesive hydrogel as NIR/pH stimulus-responsiveness platform for effectively accelerating wound healing.

Adhesive hydrogels have emerged as promising candidates to solve life-threatening infectious skin injuries. However, the inadequate mechanical characteristics and biological adherence limit the traditional wound dressing unable to adapt to high-frequency movement and real-time monitoring of wound healing, calling for the development of bioadhesive materials guided wound healing. In this work, a multifunctional bioadhesive hydrogel with double colorimetric-integrated of polyethylene glycol (PVA)-dextran (Dex)-borax-bromothymol blue (BTB)-fluorescein thiocyanate (FITC) and functionalization by tungsten disulfide-catechol nanozyme (CL/WS2) was created. Hydrogel is a perfect biological adhesive, which can achieve repeatable and strong tissue adhesion strength (8.3 ± 0.6 kPa), which is 1.66 times that of commercial dressings. Based on the strong biological adhesion of the hydrogel, a sensor is integrated into the hydrogel to collect visual image of bacterial infection from a smartphone and transform it into an on-site pH signal for remote evaluation of the wound's dynamic status in real time. Ultimately, the adhesiveness hydrogel has high worth in managing the burden related to wound healing and paving the way for intelligent wound management in the future.

Enzyme-Mimetic nano-immunosensors for amplified detection of food hazards: Recent advances and future trends.

The grave threat posed by food hazards to food security and human health has increased the urgency of rapid and sensitive detection. Enzyme-mimic immune detection, particularly nanozyme-activated food immunosensors (NAFI), shows distinct specificity and catalytic properties, advancing food safety supervision. However, knowledge of the fundamental principles and functionalities of NAFI is still limited. Based on tracking the timeline of the independent evolution and combination of immunosensors and nanozymes, we first provide a systemic view to describe the trends of NAFI. Moreover, the underlying catalytic mechanisms are discussed according to classification in this review, including metal-based, carbon-based, and metal-organic framework-based nanozymes. In particular, significant attention is focused on the fundamental function of nanozyme receptors in food immunosensors, including the connection between nanozymes and bio-affinity ligands (one pot and two steps), signal modulation and amplification, and various signal outputs (typical and novel modes), which are crucial for gaining the best performance and further innovation. Finally, based on representative applications of nanozymes to detect food hazards, the main challenges in the development of NAFI are summarized, and future research directions for this highly prospective frontier are envisioned.

A portable dual-mode colorimetric platform for sensitive detection of Hg2+ based on NiSe2 with Hg2+-Activated oxidase-like activity.

The nanozyme-based colorimetric strategy for heavy metal detection has broad application prospects nowadays. However, the inefficient recognition capabilities of nanozyme sensors for targets hinder its further application. Herein, the authors synthesize bare nickel selenide (NiSe2) via a one-step hydrothermal reaction, in which the Se element possesses a strong binding ability with mercury (Hg). As expected, NiSe2 exhibits oxidase-like activity in the presence of Hg2+, that is, Hg2+ can enhance the oxidase-like activity of NiSe2. The enhanced mechanism is the accelerated electron transfer between NiSe2-Hg2+ and substrate caused by the formation of Hg-Se bonds. Besides, the oxidase-like activity of NiSe2 exhibits excellent selectivity, sensitivity and stability in response to Hg2+, which enables NiSe2-Hg2+ to efficiently oxidize colorless TMB to blue TMB even in harsh environments. Based on this, a dual-mode colorimetric sensor integrating solution reaction and test paper is developed for the detection of Hg2+. In the Hg2+ concentration range of 10-700 nM, the colorimetric platform presents a liner response to Hg2+, which can reach a low LOD of 5.18 nM in solution reaction and 8.42 nM in the test paper. The proposed strategy can also be applied to real water samples with good recovery and excellent self-calibration capability.

Fluorescence Enhancement of Dicyanomethylene-4H-Pyran Derivatives in Solid State for Visualization of Latent Fingerprints.

The efficient development of latent fingerprint (LFP) is attractively important for criminal investigation. The low-cost and high-contrast developer is still a challenge. In this study, we designed and synthesized dicyanomethylene-4H-pyran (DCM) derivatives PZ-DCM and Boc-PZ-DCM by introducing of large steric hindrance group Boc, the solid-state fluorescence of DCM derivatives was greatly enhanced. The low-cost fluorescent LFP developers were prepared by blending with different proportion of montmorillonite (MMT). As a result, clear and high contrast fingerprint patterns were obtained with dusting method by the developer with 3% content of Boc-PZ-DCM. Furthermore, we employed the developer with 3% content of Boc-PZ-DCM to develop the sweat latent fingerprints on different substrates by powder dusting, and collected clear fingerprint patterns, indicating that the developer is universal. In a word, the Boc-PZ-DCM/MMT powder is a promising candidate for LFP developer.

Efficient and reusable photocatalytic river water disinfection by addictive graphitic carbon nitride/magnesium oxide nano-onions with particular "nano-magnifying glass effect".

Photocatalytic disinfection is a promising way to combat bacterial pollution in the water environment. Inefficient use of visible light and undirected diffusion of reactive oxygen species (ROS) reduce photocatalytic disinfection efficiency. Herein, inspired by the concentrating effect of convex lens, photocatalysts with particular "nano-magnifying glass effect" (TCNMgNOs) were designed by embedding magnesium oxide with "converge effect" into the tailored hierarchical triple-shell porous g-C3N4 with "one light multi-purpose effect" to boost the visible-light utilization. Meanwhile, the ATPase hydrolysis homeostasis of bacteria was destroyed by TCNMgNOs to achieve the targeted movement of ROS. The results confirmed that the photocatalytic sterilization efficiency of TCNMgNOs was amplified by 30 times over g-C3N4, which was achieved by focusing visible light, multiple reflecting visible light and light transmission within the porous thin shells as well as the "addictive sterilization mechanism". The sterilization efficiency still maintains 98.8 % (15 min) after 6 rounds recycling and reusing in practical river water disinfection. A novel pathway for fighting against microbial contaminants in natural water was explored.

COVID-19-inspired "artificial virus" to combat drug-resistant bacteria by membrane-intercalation- photothermal-photodynamic multistage effects.

COVID-19 threatens human life because of the super destructiveness produced from its coronal morphology and strong transmembrane infection based on spike glycoprotein. Inspired by the coronal morphology of COVID-19 and its means of infecting, we designed an "artificial virus" with coronal morphology based on the concept of "defeating superbacteria with superviruses" by self-assembling a transacting activator of transduction peptide with triple-shell porous graphitic carbon nitride (g-C3N4) embedded with cobalt nanoparticles to forcefully infect methicillin-resistant Staphylococcus aureus (MRSA). The results confirmed that this "artificial virus" had unique properties of crossing the bacterial cell membrane barrier, heating the internal bacterial microenvironment and triggering ROS outbreak, based on its coronal morphology, membrane penetration, temperature-rising and heat insulation, oxidase-like activity and excellent visible-light harvesting properties. It had a high sterilization efficiency of 99.99% at 20 min, which was 18.6 times that of g-C3N4, and the efficiency remained at 99.99% after 3 rounds of recycling and reuse. Additionally, it can rapidly inactivate bacteria in river water and accelerate wound healing.

Progress of Dicyanomethylene-4H-Pyran Derivatives in Biological Sensing Based on ICT Effect.

As one of the typical fluorescent cores, dicyanomethylene-4H-pyran (DCM) derivatives exhibit excellent photophysical and photochemical properties, such as large Stokes shift, excellent light stability, and tunable near-infrared (NIR) emission. The luminescence mechanism of DCM probes mainly depends on the intramolecular charge transfer (ICT). Hence, by regulating the ICT process, the probes can specifically act on the target molecule. Accordingly, a series of NIR DCM probes have been constructed to detect the ions, reactive oxygen species (ROS), and biological macromolecules in cells. However, there is no relevant review to summarize it at present. This minireview mainly summarizes the NIR DCM probes based on ICT effect and their applications in biosensors and biological imaging in recent years. This will be beneficial to innovatively construct new DCM probes and actively promote their application in the future.

A synergistic hydrothermal-deep eutectic solvents (DES) pretreatment for acquiring xylooligosaccharides and lignin nanoparticles from Eucommia ulmoides wood.

To achieve an effective deconstruction for preparation of xylooligosaccharides (XOS) and lignin nanoparticles (LNPs) from Eucommia ulmoides, a synergistic pretreatment was successfully developed. Herein, the hemicelluloses were preferentially dissociated in acetic acid-catalyzed hydrothermal pretreatment (HTP) for preparation of XOS, and the hydrothermally-pretreated substrate was then subjected to deep eutectic solvents (DES) delignification for fabrication of LNPs. Results showed that the optimal yield (33.88% based on xylan) of XOS is obtained under the given HTP condition (170 °C, 0.5 h). NMR characterization showed that the linkages of lignin were mainly composed of ß-O-4, ß-ß, ß-5, etc. Besides, GPC analysis showed that the molecular weight of DES lignin fractions was lower (1130-1200 g/mol) than those of corresponding parent lignin fractions (8500-9620 g/mol). Further TEM characterization indicated that the optimal LNPs fraction has a narrow size distribution and the corresponding size is ranged from 60 to 110 nm. In short, the synergistic pretreatment could be used as a green and cost-effective approach for the development of bio-based chemicals and biomaterials from Eucommia ulmoides biomass.

Structure of corn bran hemicelluloses isolated with aqueous ethanol solutions and their potential to produce furfural.

The alkali-soluble hemicelluloses extracted with 10% KOH solution from corn bran were further isolated with different concentrations of aqueous ethanol solutions. Herein 92.2% of the original hemicelluloses can be obtained and the cellulase enzymatic hydrolysis rate of the alkali treated corn bran can reach to 97.2%. The corn bran hemicelluloses were mainly glucuronoarabinoxylan, in which xylose (48.4-53.8%) and arabinose (27.8-33.2%) were the main components. More linear hemicelluloses with high molecular weight tended to be precipitated in low concentration aqueous ethanol solutions. Furthermore, the relationship between the structural features of these alkali-soluble corn bran hemicelluloses and their furfural yield was investigated in MIBK (methyl isobutyl ketone)/H2O biphasic system. Results showed that the hemicelluloses with high xylose content are benefit to the furfural production, and the highest furfural yield of 67.7% was obtained.

Graphene aerogel with excellent property prepared by doping activated carbon and CNF for free-binder supercapacitor.

In this paper, the two-step activation Eucommia wood tar-based activated carbon (ETAC), cellulose nanofibers (CNF) and reduced graphene oxide (rGO) were assembled to form composite aerogel in mild condition. Impressively, the doping of optimizing ETAC greatly improved the overall specific surface area (SSA) of the aerogel, and the CNF extracted from Eucommia ulmoides wood was used to enhance the mechanical properties of graphene aerogel. Besides, the composite aerogels with high content of ETAC (67% of mass ratio) possessed efficient MnOx deposition capability (1540 mg/g), which could assemble an asymmetric free-binder supercapacitor, exhibiting an ultrahigh specific capacitance and prominent cycling stability. This work offered a feasible method to fabricate free-binder composite aerogels with excellent electrochemical property for broad applications in supercapacitors.

Cationic Conjugated Polyelectrolytes with Aggregation-Induced Ratiometric Fluorescence.

The molecular diversity of aggregation-induced emission remains a challenge due to the limitation of conventional synthesis methods. Here, a series of novel neutral and cationic conjugated polymers composed of various ratios of tetraarylethylene (TAE) containing a bridged oxygen (O) and fluorene (F) units is designed and synthesized via the geminal cross-coupling (GCC) of 1,1-dibromoolefins. The incorporation of TAE segments into the conjugated backbone of polyfluorene produces pronounced aggregation-induced ratiometric fluorescence, i.e., aggregation-induced emission (AIE) at 520-600 nm and grows synergistically with aggregations-caused quenching (ACQ) at 400-450 nm. The content of fluorene unit in the polymer backbones determines the intensity of the initial fluorescence in the blue light region. The huge distinction (about 150 nm) in dual emission wavelengths caused by the environment change makes these conjugated polyelectrolytes particularly suitable for ratiometric fluorescence sensing. Based on electrostatic interaction mechanism, the gradual addition of heparin into the cationic conjugated polymers aqueous solutions can induce dual-color fluorescence changes with a detection limit of 9 × 10-9 m. This work exhibits the great facility of using GCC reaction to synthesis the conjugated TAE polymers with superior AIE properties and special functions.

Reversible Three-Color Fluorescence Switching of an Organic Molecule in the Solid State via "Pump-Trigger" Optical Manipulation.

In photoswitches that undergo fluorescence switching upon ultraviolet irradiation, photoluminescence and photoisomerization often occur simultaneously, leading to unstable fluorescence properties. Here, we successfully demonstrated reversible solid-state triple fluorescence switching through "Pump-Trigger" multiphoton manipulation. A novel fluorescence photoswitch, BOSA-SP, achieved green, yellow, and red fluorescence under excitation by pump light and isomerization induced by trigger light. The energy ranges of photoexcitation and photoisomerization did not overlap, enabling appropriate selection of the multiphoton light for "pump" and "trigger" photoswitching, respectively. Additionally, the large free volume of the spiropyran (SP) moiety in the solid state promoted reversible photoisomerization. Switching between "pump" and "trigger" light is useful for three-color tunable switching cell imaging, which can be exploited in programmable fluorescence switching. Furthermore, we exploited reversible dual-fluorescence switching in a single molecular system to successfully achieve two-color super-resolution imaging.

Synthesis of an environmentally friendly binding material using pyrolysis by-products and modified starch binder for slow-release fertilizers.

Biochar-based slow-release fertilizers (BSRFs) are vital for the development of eco-friendly and sustainable agriculture. Considerable attention has been given to enhancing the efficiency of fertilizers (EEFs) by appropriate modification or binding to reduce nutrient waste and improve the slow-release effect on the growth of plants. In this study, sustained binding materials were presented for BSRF synthesis, including pyroligneous acids (PA), bio-oil (BO), and modified starch binder (MSB). The results show that the release ratio of phosphorus from PA + BO+MSB was 4.7%, 15.2%, and 21.2% slower than that of PA, BO, and MSB alone, respectively. The BSRFs were characterized by SEM, XRD, FT-IR, XPS, and EDS, and the release kinetic outcome revealed that PA + BO+MSB contributed to the formation of a satisfactory structure in the BSRFs. The MSB viscosity significantly influences the slow-release performance and accumulation of N, P, and K nutrients. Moreover, economic assessments showed that PA + BO+MSB exhibited the lowest cost.

Carborane photochromism: a fatigue resistant carborane switch.

A dithienylethene molecule involving carborane clusters shows remarkable fatigue resistance and high contrast visual colour changes when irradiated with alternating ultraviolet and visible light. The fluorescence of this assembly can be switched on and off when irradiated in the solid state but not in the solution state.

Ulmoidol, an unusual nortriterpenoid from Eucommia ulmoides Oliv. Leaves prevents neuroinflammation by targeting the PU.1 transcriptional signaling pathway.

Chronic neuroinflammation is closely associated with the development of neurodegenerative diseases, including Alzheimer's disease (AD). In the current study, 13 anti-neuroinflammatory compounds were isolated from Eucommia ulmoides Oliv. leaves. Among these compounds, trans-sinapaldehyde (6), 3',4',5,7-tetrahydroxy-3-methylflavone (7), and amarusine A (13) were isolated from E. ulmoides leaves for the first time. The ursane-type C29-triterpenoid, ulmoidol (ULM, 9), significantly inhibited the production of proinflammatory mediators and reduced the expression of inducible nitric oxide synthase (iNOS) and cyclooxygenase-2 (COX-2). Moreover, ULM inhibited the cluster of differentiation 14 (CD14)/Toll-like receptor 4 (TLR4) signaling pathway and consequently limited the activation of nuclear factor kappa B (NF-κB) and mitogen-activated protein kinase (MAPK) signaling pathways. Notably, electrophoretic mobility shift assay (EMSA) and molecular docking analyses indicated that ULM could prevent PU box binding-1 (PU.1) from binding to DNA, suggesting that PU.1 might be a potential ULM target. In conclusion, ULM alleviates neuroinflammatory responses in microglia, which could be partly explained by its targeting of PU.1 and the resulting suppression of the TLR4/MAPK/NF-κB signaling pathways. These results suggested that ULM may have therapeutic potential as an agent for treating neuroinflammation-related neurodegenerative diseases.

Hydrophilic AIE-Active Tetraarylethenes for Fluorescence Sensing and Super-Resolution Imaging of Amyloid Fibrils from Hen Egg White Lysozyme.

Hen egg white lysozyme (HEWL) is frequently applied as a model protein for research on protein folding, unfolding, and fibrillization identified by featured fluorescent probes. Here, a series of hydrophilic, pH-sensitive tetraarylethene (TAE)-type AIEgens are synthesized via a geminal cross-coupling (GCC) reaction and evaluated for their capabilities of fluorescence sensing and super-resolution localization imaging of HEWL fibrils. With superior optical and sensing properties, the selected TAE-type AIEgen probe is weakly emissive in aqueous media, without dependence on the pH value and buffer concentration, but exhibits "turn-on" fluorescence upon interaction with HEWL amyloid fibrils in a spontaneous and reversible way that just meets the requirement of fluorescence random switching for super-resolution imaging. The selected probe has the strongest fluorescence response to HEWL amyloid fibrils exhibiting a limit of detection of 0.59 nmol/L and enables super-resolution fluorescence imaging of amyloid aggregates with a high resolution of 40 nm.