A Novel COL7A1 Mutation in a Patient With Dystrophic Epidermolysis Bullosa. Successful Treatment With Upadacitinib.

Dystrophic epidermolysis bullosa (DEB) is a heterogeneous and rare genetic skin disease caused by mutations in the gene, which encodes Type VII collagen. The absence or dysfunction of Type VII collagen can cause the dense lower layer of the basal membrane zone of the skin to separate from the dermis, leading to blister formation and various complications. In different DEB subtypes, the severity of the phenotype is associated, to some extent, with the outcome of Type VII collagen caused by mutations in the gene, which may be reduced in expression, remarkably reduced, or completely absent.

The influence of motivational climate on the physical activity adherence among junior high school students: The mediating effect of achievement goal orientation.

In this study, which was based on the theories of motivational climate and achievement goal orientation, an experimental intervention was conducted on 82 junior high school students using the Motivational Climate Scale (MCSYS), Task and Ego Orientation in Sport Questionnaire (TEOSQ), and Physical Activity Persistence Scale. The aim was to explore the influence of different motivational climates in physical education classes on the physical activity adherence among junior high school students and examine the mediating role of achievement goal orientation. The results revealed the following significant findings: Firstly, there was a noteworthy positive correlation between the physical activity adherence among junior high school students, the motivational climate, and achievement goal orientation.

Structural analysis and transport properties of [010]-tilt grain boundaries in Fe(Se,Te).

Understanding the nature of grain boundaries is a prerequisite for fabricating high-performance superconducting bulks and wires. For iron-based superconductors [e.g.

pH-sensitive metal-organic framework carrier decorated with chitosan for controlled drug release.

Satisfactory chemotherapeutic efficiency depends on improving the insufficient selectivity of chemotherapeutic drugs and decreasing damages to healthy tissues/cells, hence, the reasonable design and construction of drug carriers are of great significance in tumor treatment. Herein, the pH-sensitive MOF carrier was developed via the decoration of 4-formylphenylboronic acid (4-FPBA) for controlled 5'-deoxy-5-fluorocytidine (DFCR) release with selectivity. Besides, chitosan (CS) was introduced as the "gatekeeper" for the prevention of drug leakage.

Design of pH-responsive molecularly imprinted polymer as a carrier for controlled and sustainable capecitabine release.

A molecularly imprinting polymer (MIP) carrier with pH-responsivity was designed to construct a drug delivery system (DDS) focusing on controlled and sustainable capecitabine (CAPE) release. The pH-responsive characteristic was achieved by the functionalization of SiO substrate with 4-formylphenylboronic acid, accompanied by the introduction of fluorescein isothiocyanate for the visualization of the intracellular localization of the nanocarrier. Experimental results indicated that CAPE was adsorbed onto the drug carrier with satisfactory encapsulation efficiency.

Design and Algorithm Integration of High-Precision Adaptive Underwater Detection System Based on MEMS Vector Hydrophone.

Real-time DOA (direction of arrival) estimation of surface or underwater targets is of great significance to the research of marine environment and national security protection. When conducting real-time DOA estimation of underwater targets, it can be difficult to extract the prior characteristics of noise due to the complexity and variability of the marine environment. Therefore, the accuracy of target orientation in the absence of a known noise is significantly reduced, thereby presenting an additional challenge for the DOA estimation of the marine targets in real-time.

Taurine Protects against the Fatty Liver Hemorrhagic Syndrome in Laying Hens through the Regulation of Mitochondrial Homeostasis.

Metabolic-associated fatty liver disease (MAFLD) is a chronic liver disease caused by fat deposition in the liver of humans and mammals, while fatty liver hemorrhagic syndrome (FLHS) is a fatty liver disease in laying hens which can increase the mortality and cause severe economic losses to the laying industry. Increasing evidence has shown a close relationship between the occurrence of fatty liver disease and the disruption of mitochondrial homeostasis. Studies have proven that taurine can regulate hepatic fat metabolism, reduce hepatic fatty deposition, inhibit oxidative stress, and alleviate mitochondrial dysfunction.

MicroRNA-320-3p promotes the progression of acute pancreatitis by blocking DNMT3a-mediated MMP8 methylation in a targeted manner.

In this research, we screened out two genes upregulated in mice with acute pancreatitis (AP) by gene sequencing: microRNA (miR)-320-3p and matrix metalloprotease 8 (MMP8). This study was designed to determine whether miR-320-3p and MMP8 participate in AP development and explore the mechanisms, with a new idea for clinical diagnosis and treatment of AP. Expression of miR-320-3p, DNA methyltransferase 3a (DNMT3a), and MMP8 in mouse pancreatic tissues and AR42J cells was tested by RT-qPCR and western blot assays.

Polysaccharides From (Blanco) Benth.: Characterization and Antioxidant Activities.

Two polysaccharide fractions from (Blanco) Benth. () (designated as PCB-1 and PCB2-1) were isolated by water extraction and purified by Sepharose chromatography. The chemical properties of the polysaccharides were characterised, and their antioxidant activities were evaluated.

The Influence of Mixed Teaching Mode on the Learning Effects of Psychology Students' Experiment Course in Higher Vocational Colleges.

In this paper, we are going to analyze the influence of mixed teaching mode on the teaching effect of psychology students' experiment courses in higher vocational colleges. To realize and verify these claims, 80 psychology students of grade 2020 in our school were selected for observation. Class 1 of the psychology major in our school was randomly selected as the observation group ( = 40) and Class 2 as the control group ( = 40).

Rutaecarpine alleviates acute pancreatitis in mice and AR42J cells by suppressing the MAPK and NF-κB signaling pathways via calcitonin gene-related peptide.

Acute pancreatitis (AP) is an acute inflammatory condition of the pancreas. Previous studies have shown that rutaecarpine (RUT), an important alkaloid component of Evodia rutaecarpa, exhibits certain protective effects against AP in rats by upregulating calcitonin gene-related peptide (CGRP). However, the molecular mechanism of RUT in AP remains unknown.

Predictors of Mortality and Drug Resistance Among Carbapenem-Resistant Enterobacteriaceae-Infected Pancreatic Necrosis Patients.

Introduction: Carbapenem-resistant Enterobacteriaceae (CRE) has emerged as a global threat to hospitalization patients. Infected pancreatic necrosis (IPN) leads to high risks of CRE infections with increasing mortality. Our study aims to determine the predictors related to 90-day overall mortality of CRE IPN.

Analysis of gastroscopy results among healthy people undergoing a medical checkup: a retrospective study.

Background: The association of upper gastrointestinal endoscopic findings with sex, age, and Helicobacter pylori infection in asymptomatic healthy people is unclear. The aim of this study was to retrospectively determine the associations of upper gastrointestinal endoscopic findings in asymptomatic healthy people with sex, age, and H. pylori infection.

Treadmill exercise ameliorates chronic REM sleep deprivation-induced anxiety-like behavior and cognitive impairment in C57BL/6J mice.

Various sleep disorders have deleterious effects on mental and cognitive performance. Exercise, as an alternative therapeutic strategy, exerts beneficial impacts on human health. In the present study, we aimed to evaluate the effects of 4 weeks treadmill exercise (4W-TE) on anxiety-like behavior and cognitive performance in mice exposed to 2 months REM sleep deprivation (2M-SD) (20 h per day).

Emerging insights into molecular mechanisms underlying pyroptosis and functions of inflammasomes in diseases.

Pyroptosis is a form of necrotic and inflammatory programmed cell death, which could be characterized by cell swelling, pore formation on plasma membranes, and release of proinflammatory cytokines (IL-1β and IL-18). The process of pyroptosis presents as dual effects: protecting multicellular organisms from microbial infection and endogenous dangers; leading to pathological inflammation if overactivated. Two pathways have been found to trigger pyroptosis: caspase-1 mediated inflammasome pathway with the involvement of NLRP1-, NLRP3-, NLRC4-, AIM2-, pyrin-inflammasome (canonical inflammasome pathway) and caspase-4/5/11-mediated inflammasome pathway (noncanonical inflammasome pathway).