Development of a rabbit model for adrenoleukodystrophy: A pilot study on gene therapy using rAAV9.

X-linked adrenoleukodystrophy (X-ALD) is a common peroxisomal disorder caused by mutations in the gene, leading to the accumulation of very long-chain fatty acids (VLCFAs). This progressive neurodegenerative disease manifests in three primary forms: childhood-acquired cerebral demyelination (CALD), adult myelopathy (AMN), and primary adrenal cortical insufficiency. Bone marrow transplantation effectively halts disease progression only in the early stages of CALD.

Different regions and environments have critical roles on yield, main quality and industrialization of an industrial purple-fleshed sweetpotato ( L. (Lam.)) "Xuzishu8".

Purple-fleshed sweetpotato (PFSP) ( (L.) Lam), whose flesh is purple to dark purple, is a special variety type of sweetpotato, which has the characteristics of food crop, industrial crop and medicinal crop. The storage root (SR) of PFSP is rich in anthocyanins, starch, protein, soluble sugar, mineral elements, polyphenol, dietary fiber and so on, which has balanced and comprehensive nutritional value.

Effects of public health interventions and zero COVID policy on paediatric diseases: A Southern China study.

Background: With the spread of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic in schools and communities, clinical evidence is needed to determine the impact of the pandemic and public health interventions under the zero coronavirus disease policy on the occurrence of common infectious diseases and non-infectious diseases among children.

Methods: The current study was designed to analyse the occurrence of common infectious diseases before and after the pandemic outbreak in southern China. Data was obtained for 1 801 728 patients admitted into children's hospitals in Guangzhou between January 2017 and July 2022.

RNA-Sequencing Analysis Revealed Genes Associated with Sweet Potato ( (L.) Lam.) Responses to Stem Rot during Different Infection Stages.

The sweet potato, which is an important tuber crop in China, is susceptible to a variety of pathogens and insect pests during cultivation and production. Stem rot is a common sweet potato disease that seriously affects tuber yield and quality. Unfortunately, there have been relatively few studies on the mechanism mediating the stem rot resistance of sweet potatoes.

[Interaction and Transport Characteristics of Lead and Cadmium in Different Soil-wheat Systems].

In this study, four groups of lead(Pb) and cadmium(Cd) combined treatments with different concentration ratios were set up in Hailun black soil, Xianning brown-red soil, and Changwu Heilu soil, and wheat(Zhengmai 9023) was planted for a five-month pot experiment to analyze the Pb-Cd interaction behavior on heavy metal bioaccumulation in wheat under three soil-wheat systems. The low pH brown-red soil had the highest water-soluble Cd and Pb contents with significant Pb-Cd interactions in the soil, whereas the black soil with high organic matter and Heilu soil with high calcium carbonate content exhibited lower Cd and Pb activities. Among the three soils, wheat height and dry weight showed the poorest growth performance in the Heilu soil, but the wheat height increased by 2.

Comparative Analysis of Saccharification Characteristics of Different Type Sweetpotato Cultivars.

As an important characteristic crop in China, sweetpotato plays an important role in the intake and supplement of nutrients. The saccharification characteristics of sweetpotato determine the edible quality and processing type. Exploring the saccharification characteristics of sweetpotato is of great significance to the selection of processing materials and the formation mechanism of service quality, but there are few relevant studies.

Effects of excitation power density on the Stern-Volmer constant measurement.

The Stern-Volmer constant (K) is an important parameter to describe the capability of energy transfer to oxygen for porphyrin and its derivatives. By fitting Stern-Volmer equation, I/I= 1 + K[O], the K is generally determined through phosphorescence intensities (I) under aerobic and oxygen-free conditions. In this work, the effect of excitation power density on the K measurement is theoretically analyzed and experimentally studied, using palladium octaethylporphyrin (PdOEP) as an example.

Experimental Verification of the Accuracy of Oxygen-Varying Correlated Fluorescence for Determining the Quenching Constant .

The oxygen-varying correlated fluorescence provides an effective strategy to gain the quenching constant for a non-phosphorescent system. Owing to the absence of detectable phosphorescence emission in general optical spectroscopy, the facticity of this method has never been verified. Here, the correct way to determine by oxygen-varying correlated fluorescence was systematically studied in detail.

Accurate Determination of the Photosensitizer Stern-Volmer Constant by the Oxygen-Dependent Consumption of 1,3-Diphenylisobenzofuran.

Because of the absence of phosphorescence, the Stern-Volmer constant () of the photosensitizer is hard to determine accurately. Although the delayed fluorescence and correlated fluorescence methods have been proposed to determine , the weak signal detection and non-uniform excitation enlarged the measurement error. In this work, a method was proposed to accurately determine by oxygen-dependent consumption of 1,3-diphenylisobenzofuran.

Bradykinin-pretreated Human cardiac-specific c-kit Cells Enhance Exosomal miR-3059-5p and Promote Angiogenesis Against Hindlimb Ischemia in mice.

Background: Protection of cardiac function following myocardial infarction was largely enhanced by bradykinin-pretreated cardiac-specific c-kit (BK-c-kit) cells, even without significant engraftment, indicating that paracrine actions of BK-c-kit cells play a pivotal role in angiogenesis. Nevertheless, the active components of the paracrine actions of BK-c-kit cells and the underlying mechanisms remain unknown. This study aimed to define the active components of exosomes from BK-c-kit cells and elucidate their underlying protective mechanisms.

DWL-4-140: A allene small molecule targeting STING that alleviates lupus-like phenotype in Trex1 mice.

The innate immune system plays a critical role in the host response against pathogenic microbial infection. However, aberrant activation of the innate immune pathways is a characteristic feature of various diseases. Thus, targeted drugs must be developed based on the understanding of the innate immune signaling pathways.

A New Insight into the Singlet Oxygen Mechanism for Photodynamic Therapy.

Modern photodynamic therapy has been built on the mechanism of the interaction between the photosensitizer (porphyrin derivatives) and oxygen to produce singlet oxygen, which relies on energy transfer from the triplet excited state (T) of porphyrin to the excited state of oxygen. In this process, the energy transfer from the singlet excited state (S) of porphyrin to oxygen is believed to be not pronounced as the rapid decay of S and the large energy mismatch. Here, we have evidenced the existence of an energy transfer between S and oxygen, which can contribute to the production of singlet oxygen.

Identification and functional characterization of a flavonol synthase gene from sweet potato [ (L.) Lam.].

Flavonol synthase (FLS) is a key enzyme of the flavonoid biosynthetic pathway, which catalyzes the conversion of dihydroflavonols into flavonols. In this study, the FLS gene was cloned and characterized from sweet potato. The resulting IbFLS1 protein showed a high similarity with other plant FLSs.

Quantitative analysis of dose interval effect of Pb-Cd interaction on Oryza sativa L. root.

Combined pollution of cadmium (Cd) and lead (Pb) occurs frequently in agriculture lands, which has received increasing research attention. However, little is known about the interaction behaviors of Cd and Pb at various concentrations in the mixture. This study evaluated the single and combined effects of Cd and Pb on rice (Oryza sativa L.

Insight into the Heavy Atom Effect Induced by Environmental Heavy Atoms for Gadolinium-Labeled Hematoporphyrin Monomethyl Ether.

Environmental heavy atoms can further enhance the room temperature phosphorescence (RTP) emissions of gadolinium-labeled hematoporphyrin monomethyl ether (Gd-HMME) by way of the external heavy atom effect (HAE). However, the macroscopic phosphorescence intensity covered the intrinsic effect of the environmental heavy atoms. In this study, a method of separating the external HAE from the total is performed, and a quantity to describe the intrinsic nature of external HAE is defined.

Mitochondrial Cardiomyopathy: Molecular Epidemiology, Diagnosis, Models, and Therapeutic Management.

Mitochondrial cardiomyopathy (MCM) is characterized by abnormal heart-muscle structure and function, caused by mutations in the nuclear genome or mitochondrial DNA. The heterogeneity of gene mutations and various clinical presentations in patients with cardiomyopathy make its diagnosis, molecular mechanism, and therapeutics great challenges. This review describes the molecular epidemiology of MCM and its clinical features, reviews the promising diagnostic tests applied for mitochondrial diseases and cardiomyopathies, and details the animal and cellular models used for modeling cardiomyopathy and to investigate disease pathogenesis in a controlled in vitro environment.

Genome-Wide Identification of the A20/AN1 Zinc Finger Protein Family Genes in and Its Two Relatives and Function Analysis of in Salinity Tolerance.

Stress-associated protein (SAP) genes-encoding A20/AN1 zinc-finger domain-containing proteins-play pivotal roles in regulating stress responses, growth, and development in plants. They are considered suitable candidates to improve abiotic stress tolerance in plants. However, the gene family in sweetpotato () and its relatives is yet to be investigated.

Intrinsic Characterization Method on the Heavy Atom Effect of Metalloporphyrins.

The presence of room temperature phosphorescence emission in metalloporphyrin, via the transition from the excited triplet state (T) to the ground state (S), relies on the chelated heavy metal ions, which is known as the heavy atom effect (HAE). Despite the HAE being a reliable method to tune the phosphorescence process widely, the realization of the HAE nature is a tough task as the induced phosphorescence process is sensitive to not only the specie of bonded heavy atoms but also chemical environments such as the oxygen quenching and solvent effect. In this study, we have aimed at a quantitative determination of the intrinsic phosphorescent transition rate () in metalloporphyrin gadolinium-labeled hematoporphyrin monomethyl ether (Gd-HMME).

A C2-Domain Abscisic Acid-Related Gene, , Positively Enhances Salt Tolerance in Sweet Potato ( (L.) Lam.).

Plant C2-domain abscisic acid-related (CAR) protein family plays an important role in plant growth, abiotic stress responses, and defense regulation. In this study, we cloned the by homologous cloning method from the transcriptomic data of Xuzishu8, which is a sweet potato cultivar with dark-purple flesh. This gene was expressed in all tissues of sweet potato, with the highest expression level in leaf tissue, and it could be induced by NaCl and ABA.

Topical and intravenous administration of human umbilical cord mesenchymal stem cells in patients with diabetic foot ulcer and peripheral arterial disease: a phase I pilot study with a 3-year follow-up.

Background: Diabetic foot ulcer (DFU) is a serious chronic complication of diabetes mellitus that contributes to 85% of nontraumatic lower extremity amputations in diabetic patients. Preliminary clinical benefits have been shown in treatments based on mesenchymal stem cells for patients with DFU or peripheral arterial disease (PAD). However, the long-term safety and benefits are unclear for patients with both DFU and PAD who are not amenable to surgical revascularization.

Evaluation of Physiological Coping Strategies and Quality Substances in Purple SweetPotato under Different Salinity Levels.

Although salinity stress is one of the principal abiotic stresses affecting crop yield, a suitable concentration of NaCl has proven to be useful for increasing crop quality. This study used low salinity (34 mmol/L NaCl) and high salinity (85 mmol/L) to cultivate purple sweetpotato. Using transcriptomics and metabolomics to profile the pathway indicated that glycometabolism, secondary metabolite biosynthesis and the starch catabolic process were the significant pathways under the salinity stress.

Comparative Transcriptome Profiling Reveals the Genes Involved in Storage Root Expansion in Sweetpotato ( (L.) Lam.).

Sweetpotato ( (L.) Lam.) is recognized as one of the most important root crops in the world by the Food and Agriculture Organization of the United Nations.

Mesenchymal stem cell-derived extracellular vesicles for immunomodulation and regeneration: a next generation therapeutic tool?

Mesenchymal stem cells (MSCs) can be widely isolated from various tissues including bone marrow, umbilical cord, and adipose tissue, with the potential for self-renewal and multipotent differentiation. There is compelling evidence that the therapeutic effect of MSCs mainly depends on their paracrine action. Extracellular vesicles (EVs) are fundamental paracrine effectors of MSCs and play a crucial role in intercellular communication, existing in various body fluids and cell supernatants.