Diagnostic value of nerve conduction study in NOTCH2NLC-related neuronal intranuclear inclusion disease.

BACKGROUND AND AIMS: Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disorder mainly caused by abnormally expanded GGC repeats within the NOTCH2NLC gene. Most patients with NIID show polyneuropathy. Here, we aim to investigate diagnostic electrophysiological markers of NIID. METHODS: In this retrospective dual-center study, we reviewed 96 patients with NOTCH2NLC-related NIID, 94 patients with genetically confirmed Charcot-Marie-Tooth (CMT) disease, and 62 control participants without history of peripheral neuropathy, who underwent nerve conduction studies between 2018 and 2022. RESULTS: Peripheral nerve symptoms were presented by 53.1% of patients with NIID, whereas 97.9% of them showed peripheral neuropathy according to electrophysiological examinations. Patients with NIID were characterized by slight demyelinating sensorimotor polyneuropathy; some patients also showed mild axonal lesions. Motor nerve conduction velocity (MCV) of the median nerve usually exceeded 35 m/s, and were found to be negatively correlated with the GGC repeat sizes. Regarding the electrophysiological differences between muscle weakness type (n = 27) and non-muscle weakness type (n = 69) of NIID, nerve conduction abnormalities were more severe in the muscle weakness type involving both demyelination and axonal impairment. Notably, specific DWI subcortical lace sign was presented in only 33.3% of muscle weakness type, thus it was difficult to differentiate them from CMT. Combining age of onset, distal motor latency, and compound muscle action potential of the median nerve showed the optimal diagnostic performance to distinguish NIID from major CMT (AUC = 0.989, sensitivity = 92.6%, specificity = 97.4%). INTERPRETATION: Peripheral polyneuropathy is common in NIID. Our study suggest that nerve conduction study is useful to discriminate NIID.

Natural polyphenols for drug delivery and tissue engineering construction: A review.

Polyphenols, natural compounds rich in phenolic structures, are gaining prominence due to their antioxidant, anti-inflammatory, antibacterial, and anticancer properties, making them valuable in biomedical applications. Through covalent and noncovalent interactions, polyphenols can bind to biomaterials, enhancing their performance and compensating for their shortcomings. Such polyphenol-based biomaterials not only increase the efficacy of polyphenols but also improve drug stability, control release kinetics, and boost the therapeutic effects of drugs. They offer the potential for targeted drug delivery, reducing off-target impacts and enhancing therapeutic outcomes. In tissue engineering, polyphenols promote cell adhesion, proliferation, and differentiation, thus aiding in the formation of functional tissues. Additionally, they offer excellent biocompatibility and mechanical strength, essential in designing scaffolds. This review explores the significant roles of polyphenols in tissue engineering and drug delivery, emphasizing their potential in advancing biomedical research and healthcare.

Pharmaceutical applications of chitosan in skin regeneration: A review.

Skin regeneration is the process that restores damaged tissues. When the body experiences trauma or surgical incisions, the skin and tissues on the wound surface become damaged. The body repairs this damage through complex physiological processes to restore the original structural and functional states of the affected tissues. Chitosan, a degradable natural bioactive polysaccharide, has attracted widespread attention partly owing to its excellent biocompatibility and antimicrobial properties; additionally, a modified form of this compound has been shown to promote skin regeneration. This review evaluates the recent research progress in the application of chitosan to promote skin regeneration. First, we discuss the basic principles of the extraction and preparation processes of chitosan from its source. Subsequently, we describe the functional properties of chitosan and the optimization of these properties through modification. We then focus on the existing chitosan-based biomaterials developed for clinical applications and their corresponding effects on skin regeneration, particularly in cases of diabetic and burn wounds. Finally, we explore the challenges and prospects associated with the use of chitosan in skin regeneration. Overall, this review provides a reference for related research and contributes to the further development of chitosan-based products in cutaneous skin regeneration.

Evaluating the hypolipidemic effect of garlic essential oil encapsulated in a novel double-layer delivery system.

The limited application of garlic essential oil (GEO) is attributed to its pungent taste, poor water solubility and low bioavailability. Liposomes are nontoxic, biodegradable and biocompatible, and ß-cyclodextrin can inhibit undesirable odors and improve the stability and bioavailability. Thus a promising dual-layer GEO ß-cyclodextrin inclusion compound liposome (GEO-DCL) delivery system with both advantages was designed and prepared in this study. Experimental results indicated that the encapsulation efficiency of GEO-DCLs was 5% higher than that of GEO liposomes (GEO-CLs), reaching more than 88%. In vitro release experiment showed that the release rate of GEO in GEO-DCLs was 40% lower than that of GEO-CLs after incubation in gastric juice for 6-h, indicating that the stability of GEO-DCLs was better than GEO-CLs. Evaluation of the effects of GEO-DCLs on lowering blood lipid levels in hypercholesterolemia mice. GEO-DCLs could reduce the weight and fat deposition in hypercholesterolemia mice. Inhibiting the increase of TC, LDL-C, and decrease of HDL-C in mice. The degree of liver injury was decreased, the number of round lipid droplets in liver cytoplasm was reduced, and the growth of fat cells was inhibited. The lipid-lowering effects of GEO-DCLs were dose-dependent. GEO-DCL can improve the bioavailability of GEO and improve dyslipidemia. Based on GEO's efficacy in lowering blood lipids, this study developed a kind of GEO-DCL compound pomegranate juice beverage with good taste, miscibility and double effect of reducing blood lipids. This study lays a foundation for the application of GEO in the field of functional food.

A molecularly imprinted polymer with incorporated graphene oxide for electrochemical determination of quercetin.

The molecularly imprinted polymer based on polypyrrole film with incorporated graphene oxide was fabricated and used for electrochemical determination of quercetin. The electrochemical behavior of quercetin on the modified electrode was studied in detail using differential pulse voltammetry. The oxidation peak current of quercetin in B-R buffer solution (pH = 3.5) at the modified electrode was regressed with the concentration in the range from 6.0 × 10(-7) to 1.5 × 10(-5) mol/L (r2 = 0.997) with a detection limit of 4.8 × 10(-8) mol/L (S/N = 3). This electrode showed good stability and reproducibility. In the above mentioned range, rutin or morin which has similar structures and at the same concentration as quercetin did not interfere with the determination of quercetin. The applicability of the method for complex matrix analysis was also evaluated.

Causal effects of gut microbiota on the risk of periodontitis: a two-sample Mendelian randomization study.

Introduction: The oral cavity and the gut tract are interconnected, and both contain abundant natural microbiota. Gut microbiota may interact with oral flora and participate in the development of periodontitis. However, the specific role of certain gut microbiota taxa for periodontitis has not been investigated. Mendelian Randomization is an ideal method to explore causal relationships avoiding reverse causality and potential confounding factors. Thus, we conducted a two-sample Mendelian Randomization study to comprehensively reveal the potential genetic causal effect of gut microbiota on periodontitis. Methods: SNPs strongly associated with 196 gut microbiota taxa (18,340 individuals) were selected as instrument variables, and periodontitis (17,353 periodontitis cases and 28,210 controls) was used as the outcome. The causal effect was analyzed via random effect inverse variance-weighted, weighted median, and MR-Egger. The sensitivity analyses were conducted using Cochran's Q tests, funnel plots, leave-one-out analyses, and MR-Egger intercept tests. Results: Nine gut microbiota taxa (Prevotella 7, Lachnospiraceae UCG-008, Enterobacteriales, Pasteurellales, Enterobacteriaceae, Pasteurellaceae, Bacteroidales S24.7 group, Alistipes, and Eisenbergiella) are predicted to play a causal role in enhancing the risk of periodontitis (p< 0.05). Besides, two gut microbiota taxa (Butyricicoccus and Ruminiclostridium 6) have potentially inhibitive causal effects on the risk of periodontitis (p< 0.05). No significant estimation of heterogeneity or pleiotropy is detected. Conclusion: Our study demonstrates the genetic causal effect of 196 gut microbiota taxa on periodontitis and provides guidance for the clinical intervention of periodontitis.

Chromosome-level genome of butterflyfish unveils genomic features of unique colour patterns and morphological traits.

Chaetodontidae, known as butterflyfishes, are typical fish in coral ecosystems, exhibiting remarkable interspecific differences including body colour patterns and feeding ecology. In this study, we report genomes of three butterflyfish species (Chelmon rostratus, Chaetodon trifasciatus and Chaetodon auriga) and a closely related species from the Pomacanthidae family, Centropyge bicolour, with an average genome size of 65,611 Mb. Chelmon rostratus, comprising 24 chromosomes assembled to the chromosome level, could be served as a reference genome for butterflyfish. By conducting a collinearity analysis between butterflyfishes and several fishes, we elucidated the specific and conserved genomic features of butterflyfish, with particular emphasis on novel genes arising from tandem duplications and their potential functions. In addition to the two melanocyte-specific tyr genes commonly found in fish, we found the gene tyrp3, a new tyrosinase-related proteins gene in the reef fish, including butterflyfish and clownfish, implicating their involvement in the pigmentation diversity of fish. Additionally, we observed a tandem duplication expansion of three copies of nell1 gene in C. rostratus genome, which likely contribute to its unique jaw development and distinctive morphology of its sharp mouth. These results provided valuable genomic resources for further investigations into the genetic diversity and evolutionary adaptations of reef fish.

Biological Safe Gold Nanoparticle-Modified Dental Aligner Prevents the Porphyromonas gingivalis Biofilm Formation.

Oral microbiology could directly influence overall health. Porphyromonas gingivalis (P. gingivalis) is a highly pathogenic bacterium that causes periodontitis and other related systematic diseases, including Alzheimer's disease. Orthodontic devices (e.g., invisalign aligner) is commonly used in populations with periodontitis who are also at a high risk of systematic diseases. In this study, newly explored antibacterial 4,6-diamino-2-pyrimidinethiol-modified gold nanoparticles (AuDAPT) were coated onto aligners. The coated aligners showed favorable antibacterial activity against P. gingivalis. In the presence of the coated aligner, the number of planktonic cells was decreased, and biofilm formation was prevented. This material also showed favorable biocompatibility in vivo and in vitro. This study reveals a new method for treating oral P. gingivalis by coating aligners with AuDAPT, which has typical advantages compared to other treatments for both periodontitis and related systematic diseases.

Gold Nanoclusters-Coated Orthodontic Devices Can Inhibit the Formation of Streptococcus mutans Biofilm.

Oral health is an issue that has attracted increasing attention recently. Poor oral hygiene may induce the formation of oral biofilm on orthodontic devices, and cause gingivitis and dental caries. Here, we present a strategy for modifying orthodontic devices (e.g., invisalign aligner) with quaternary ammonium (QA)-modified gold nanoclusters (QA-GNCs) as an antibiotic reagent to prevent bacterial contamination and biofilm formation. The QA-GNCs-coated aligner can efficiently inhibit the adhesion of cariogenic pathogenic Streptococcus mutans and the formation of biofilm. Moreover, the antibacterial activity of the coated QA-GNCs can be maintained for at least 3 months and after repeated usage (>3 cycles). Furthermore, the QA-GNCs coating shows excellent biosafety confirmed by the cell viability test, the hemolysis assay, and animal experiments. Our strategy for antibacterial coating has the advantages of broad applications, low cost, good stability, high antibacterial efficiency, good biocompatibility, and low risk of antibiotic contamination, which could be particularly useful in preventing infections involving implantable medical devices or wearable electronics.

Neurological complications and risk factors of cardiopulmonary failure of EV-A71-related hand, foot and mouth disease.

From 2010 to 2012, large outbreaks of EV-A71-related- hand foot and mouth disease (HFMD) occurred annually in China. Some cases had neurological complications and were closely associated with fatal cardiopulmonary collapse, but not all children with central nervous system (CNS) involvement demonstrated a poor prognosis. To identify which patients and which neurological complications are more likely to progress to cardiopulmonary failure, we retrospectively studied 1,125 paediatric inpatients diagnosed with EV-A71-related HFMD in Hunan province, including 1,017 cases with CNS involvement. These patients were divided into cardiopulmonary failure (976 people) group and group without cardiopulmonary failure (149 people). A logistic regression analysis was used to compare the clinical symptoms, laboratory test results, and neurological complications between these two groups. The most significant risk factors included young age, fever duration ≥3 days, coma, limb weakness, drowsiness and ANS involvement. Patients with brainstem encephalitis and more CNS-involved regions were more likely to progress to cardiopulmonary failure. These findings can help front-line clinicians rapidly and accurately determine patient prognosis, thus rationally distributing the limited medical resources and implementing interventions as early as possible.