Genetic and clinical spectrum of steroid-resistant nephrotic syndrome with nuclear pore gene mutation.

Background: Steroid-resistant nephrotic syndrome (SRNS) is insensitive to steroid therapy and overwhelmingly progresses to kidney failure (KF), the known pathogenic genes of which include key subunits of the nuclear pore complex (NPC), a less-recognized contributor to glomerular podocyte injury.

Methods: After analyzing their clinical characterizations and obtaining parental consent, whole-exome sequencing (WES) was performed on patients with SRNS. Several nucleoporin (NUP) biallelic pathogenic variants were identified and further analyzed by cDNA-PCR sequencing from white cells of peripheral blood, minigene assay, immunohistochemical (IHC) staining, and electron microscopy (EM) ultrastructure observation of kidney biopsy, as well as multiple in silico prediction tools, including 3D protein modeling.

Moderately mechanically activated starch in improving protein digestibility: Application in noodles.

The aim of this study was to investigate the mechanism of protein digestibility improvement by exploring the changes in structural characteristics of proteins in noodles with varying levels of mechanically activated starch. Therefore, different levels of mechanically activated wheat starch were mixed with refined wheat flour to produce noodles. Results showed that moderately mechanically activated starch could significantly improve protein digestibility and noodles containing 8.

Two-stage photodegradation of indomethacin molecular nanocomposites under extreme confinement.

The incorporation of a glassy material into a self-assembled nanoparticle (NP) film can produce highly loaded nanocomposites. Reduction of the NP diameter can lead to extreme nanoconfinement of the glass, significantly affecting the thermal and physical properties of the nanocomposite material. Here, we investigate the photostability and photodegradation mechanisms of molecular nanocomposite films (MNCFs) produced from the infiltration of indomethacin (IMC) molecules into self-assembled films of silica NPs (11-100 nm in diameter).

Fe diaspora titanium dioxide and graphene: A study of conductive powder materials and coating applications.

Developing new conductive primers to ensure electrostatic spraying is crucial in response to the call for lightweight production of new energy vehicles. We report a stabilized material, Fe-T/G, of Fe-doped TiO composite graphene synthesized by a simple hydrothermal and electrostatic self-assembly method. The resistivity decreases from 0.

Molecular Dynamics Study on Thermal Conductivity Properties and Dielectric Behaviors of Graphene-Based Epoxy Resin Nanocomposites.

In order to increase the thermal conductivity of neat epoxy resin and broaden its practical application in high-voltage insulation systems, we have constructed four kinds of epoxy resin nanocomposite models (a neat epoxy resin (EP), a graphene-doped epoxy resin nanocomposite (EP/GR) and hydroxyl- or carboxyl-functionalized graphene-doped epoxy resin nanocomposites (EP/GR-OH or EP/GR-COOH)) to systematically investigate their thermodynamic and electrical properties using molecular dynamics (MD) simulations. Compared with the EP model, carboxyl-functionalized graphene particles enhanced the thermal conductivity of the EP/GR-COOH model by 66.5% and increased its by 26.

Safety and immunogenicity of an optimized self-replicating RNA platform for low dose or single dose vaccine applications: a randomized, open label Phase I study in healthy volunteers.

Self-replicating RNA (srRNA) technology, in comparison to mRNA vaccines, has shown dose-sparing by approximately 10-fold and more durable immune responses. However, no improvements are observed in the adverse events profile. Here, we develop an srRNA vaccine platform with optimized non-coding regions and demonstrate immunogenicity and safety in preclinical and clinical development.

Dynamic Boronic Ester Cross-Linked Polymers with Tunable Properties via Side-Group Engineering.

The development of dynamic covalent materials with repairability, reprocessability, and recyclability is crucial for sustainable development. In this work, we report a new strategy to adjust the thermomechanical properties of boronic ester cross-linked poly(β-hydroxyl amine)s through side-group engineering. By tuning the side groups of the poly(β-hydroxyl amine)s, we have developed self-healable, reprocessable, and shape-programmable materials.

Title: Identification of a novel GRHPR mutation in primary hyperoxaluria type 2 and establishment of patient-derived iPSC line.

This research delves into Primary Hyperoxaluria Type 2 (PH2), an autosomal recessive disorder precipitated by a unique case of compound heterozygous deleterious mutations in the GRHPR gene, specifically the intron2/3 c.214-2 T > G and the exon8 c.864-865delTG, leading to a premature stop codon at p.

Morphological Characteristics, Mitochondrial Genome, and Evolutionary Insights Into a New Sea Squirt From the Beibu Gulf.

A new species of the genus Microcosmus was described in this study based on specimens collected from the coast of Xilian Town, Xuwen County, Zhanjiang, Guangdong Province, China. The morphological and molecular characteristics of this new species, sp. z YZ-2024 (YZ-2024), distinguish it from other sea squirts.

Comprehensive Mass Spectral Libraries of Human Thyroid Tissues and Cells.

Thyroid nodules are a common endocrine condition with an increasing incidence over the decades. Data-independent acquisition has been widely utilized in discovery proteomics to identify disease biomarkers and therapeutic targets. To analyze the thyroid disease-related proteome in a high-throughput, reproducible and reliable manner, we introduce thyroid-specific peptide spectral libraries.

AI-driven eyelid tumor classification in ocular oncology using proteomic data.

Eyelid tumors pose diagnostic challenges due to their diverse pathological types and limited biopsy materials. This study aimed to develop an artificial intelligence (AI) diagnostic system for accurate classification of eyelid tumors. Utilizing mass spectrometry-based proteomics, we analyzed proteomic data from eight tissue types and identified eighteen novel biomarkers based on 233 formalin-fixed, paraffin-embedded (FFPE) samples from 150 patients.

Acute systemic macrophage depletion in osteoarthritic mice alleviates pain-related behaviors and does not affect joint damage.

Background: Osteoarthritis (OA) is a painful degenerative joint disease and a leading source of years lived with disability globally due to inadequate treatment options. Neuroimmune interactions reportedly contribute to OA pain pathogenesis. Notably, in rodents, macrophages in the DRG are associated with onset of persistent OA pain.

Effects of Ethylene Propylene Diene Monomer (EPDM)-Based Polar Macromolecular Compatibilizers on the Low-Temperature Properties of Fluoroelastomer/EPDM Rubber Blends.

Integrating rubber with superior low-temperature capabilities, such as ethylene propylene diene monomer (EPDM), is a strategic approach to bolster the low-temperature performance of fluoroelastomer (FKM). However, FKM and EPDM are thermodynamically incompatible. This work synthetized three EPDM-based polar macromolecular compatibilizers, epoxidized EPDM (EPDM-EP), 2,2-trifluoroethylamine-grafted epoxidized EPDM (EPDM-TF), and 2,4-difluorobenzylamine-grafted epoxidized EPDM (EPDM-DF), to enhance the compatibility between FKM and EPDM.

π-HuB: the proteomic navigator of the human body.

The human body contains trillions of cells, classified into specific cell types, with diverse morphologies and functions. In addition, cells of the same type can assume different states within an individual's body during their lifetime. Understanding the complexities of the proteome in the context of a human organism and its many potential states is a necessary requirement to understanding human biology, but these complexities can neither be predicted from the genome, nor have they been systematically measurable with available technologies.

Metal-Organic Coordination Enhanced Metallopolymer Electrolytes for Wide-Temperature Solid-State Lithium Metal Batteries.

The practical application of polymer electrolytes is seriously hindered by the inferior Li ionic conductivity, low Li transference number (t), and poor interfacial stability. Herein, a structurally novel metallopolymer is designed and synthesized by exploiting a molybdenum (Mo) paddle-wheel complex as a tetratopic linker to bridge organic and inorganic moieties at molecular level. The prepared metallopolymer possesses combined merits of outstanding mechanical and thermal stability, as well as a low glass transition temperature (T <-50 °C).

Construction of an In Situ-Generated Nanoscale Organic-Inorganic Hybrid System Using Hydrogen Bonding Interaction To Assist Thermal Properties of Resins.

With the rapid development of science and technology, high-temperature-resistant resin systems are facing more severe challenges in extreme applications. To further improve the comprehensive thermal properties of phthalonitrile resins, an in situ generation of a high-temperature-resistant phthalonitrile resin achieving an organic-inorganic hybridization network is reported. A 3-aminophenol phthalonitrile containing -NH is used as a material to hybridize with prepared calcium phosphate nano-oligomers (CPOs), and the hybrid precursor is named as CAPN.

Tailoring tetrahedral and pair-correlation entropies of glass-forming liquids for energy storage applications at ultralow temperatures.

Aqueous solution experiences either crystallization or vitrification as being cooled, yet the mechanism of this bifurcation is confused. Since the glass-transition temperature T is much lower than the melting temperature, we herein propose an entropy-driven glass-forming liquid (EDGFL) as an attractive concept to develop anti-freezing electrolytes. The T is delicately modulated via regulating local structural orders to avoid the energy-driven ice crystallization and enter an entropy-driven glass transition, which can be theoretically explained by the competitive effect between tetrahedral entropy of water and pair correlation entropy related to ions.

Effects of different ratios of water and glycerol on the physicochemical properties of starch-based straws.

This study aimed to assess the effects of different ratios of water and glycerol on the physicochemical properties of starch-based straws (SBS) and explore their plasticising mechanism. X-ray diffraction analysis revealed that the V-type complex formation was influenced by adjusting the ratio of glycerol and water. The extruded samples exhibited lower water content (4.

PGC-1α drives small cell neuroendocrine cancer progression toward an ASCL1-expressing subtype with increased mitochondrial capacity.

Adenocarcinomas from multiple tissues can converge to treatment-resistant small cell neuroendocrine (SCN) cancers composed of ASCL1, POU2F3, NEUROD1, and YAP1 subtypes. We investigated how mitochondrial metabolism influences SCN cancer (SCNC) progression. Extensive bioinformatics analyses encompassing thousands of patient tumors and human cancer cell lines uncovered enhanced expression of proliferator-activatedreceptor gamma coactivator 1-alpha (PGC-1α), a potent regulator of mitochondrial oxidative phosphorylation (OXPHOS), across several SCNCs.

Generation, Characterization, and Preclinical Studies of a Novel NKG2A-Targeted Antibody BRY805 for Cancer Immunotherapy.

Immuno-oncology has revolutionized cancer treatment, with NKG2A emerging as a novel target for immunotherapy. The blockade of NKG2A using the immune checkpoint inhibitor (ICI) monalizumab has been shown to enhance the responses of both NK cells and CD8+ T cells. However, monalizumab has demonstrated limited efficacy in in vitro cytotoxic assays and clinical trials.

Melatonin treatment increases skin microbiota-derived propionic acid to alleviate atopic dermatitis.

Background: Melatonin has been reported to relieve the inflammatory symptoms and improve sleep disturbance in patients with atopic dermatitis (AD). Recent studies showed that melatonin produced beneficial effects by remodeling intestinal microbiota composition; however, whether the beneficial effects of melatonin in AD were mediated by the modulation of skin microbiota remains unclear.

Objective: We sought to investigate the mechanism by which melatonin treatment-induced changes in the skin microbiota composition further alleviated AD.

Enhancement of far-infrared laser damage threshold in Ge-As-Se glass fiber.

The application of As-Se chalcogenide glasses in high-power laser delivery is hindered by their low damage threshold due to their weak chemical bonds. To solve this, we introduced germanium elements into the As-Se glasses and optimized the composition to raise the glass transition temperature (T) and enhance the laser damage threshold (LDT). From the correlation among various parameters including T, LDT, and fiber loss, we concluded an optimized composition of GeAsSe/GeAsSe glass.