Palmitoylation acts as a checkpoint for MAVS aggregation to promote antiviral innate immune responses.

Upon RNA virus infection, the signaling adaptor MAVS forms functional prion-like aggregates on the mitochondrial outer membrane, which serve as a central hub that links virus recognition to downstream antiviral innate immune responses. Multiple mechanisms regulating MAVS activation have been revealed; however, the checkpoint governing MAVS aggregation remains elusive. Here, we demonstrated that the palmitoylation of MAVS at cysteine 79 (C79), which is catalyzed mainly by the palmitoyl S-acyltransferase ZDHHC12, was essential for MAVS aggregation and antiviral innate immunity upon viral infection in macrophages.

Hypermethioninemia due to methionine adenosyltransferase I/III deficiency and brain damage.

Background And Objectives: Methionine adenosyltransferase I/III deficiency used to be considered a relatively benign disease. This study aims to elucidate the clinical characteristics of methionine adenosyltransferase I/III deficiency patients with neurological manifestations.

Methods: The clinical data, blood amino acids, plasma total homocysteine, gene variants, brain imaging, treatments and outcomes of 15 patients with methionine adenosyltransferase I/III deficiency were retrospectively analyzed.

Dissociation of Nicotine from Acetylcholine-Binding Protein under Terahertz Waves Radiation.

The binding of nicotine (NCT) to acetylcholine-binding protein (AChBP) plays an important role in synaptic transmission and neurotransmitter regulation. However, effectively regulating their binding or dissociation processes remains a challenging problem. In this study, we employed all-atom molecular dynamics (MD) simulations to systematically investigate the impact of external terahertz (THz) waves on the binding kinetics between AChBP and NCT.

A flexible resistive strain gauge with reduced temperature effect via thermal expansion anisotropic composite substrate.

Strain gauge plays vital roles in various fields as structural health monitoring, aerospace engineering, and civil infrastructure. However, traditional flexible strain gauge inevitably brings the pseudo-signal caused by the substrate temperature effect and determines its accuracy. Here, we present an anisotropic composite substrate designed to modify the thermal expansion performance via Micro-electro-mechanical System (MEMS) technology, which facilitates the development of strain gauges that are minimally affected by substrate temperature-induced effect.

Clinical features and CPS1 variants in Chinese patients with carbamoyl phosphate synthetase 1 deficiency.

Background: Carbamoyl phosphate synthetase 1 (CPS1) deficiency (OMIM 237300), an autosomal recessive rare and severe urea cycle disorder, is associated with hyperammonemia and high mortality.

Methods: Herein we present 12 genetic variants identified in seven clinically well-characterized Chinese patients with CPS1 deficiency who were admitted to the Children's Medical Center of Peking University First Hospital from September 2014 to August 2023.

Results: Seven patients (two male and five female patients including two sisters) experienced symptoms onset between 2 days and 13 years of age, and they were diagnosed with CPS1 deficiency between 2 months and 20 years.

The Protective Effect of Vitexin on Hypertensive Nephropathy Rats.

Introduction: Vitexin is a natural flavonoid compound extracted from Vitex leaves or seeds, exhibiting various pharmacological activities including anticancer, antihypertensive, anti-inflammatory, and spasmolytic effects. However, its protective effects on hypertensive nephropathy (HN) and the underlying mechanisms remain unclear.

Methods: Spontaneous hypertension rats were fed a high-sugar and high-fat diet for 8 weeks to induce the disease HN model.

The utility of methylmalonic acid, methylcitrate acid, and homocysteine in dried blood spots for therapeutic monitoring of three inherited metabolic diseases.

Backgroud: Routine metabolic assessments for methylmalonic acidemia (MMA), propionic acidemia (PA), and homocysteinemia involve detecting metabolites in dried blood spots (DBS) and analyzing specific biomarkers in serum and urine. This study aimed to establish a liquid chromatography-tandem mass spectrometry (LC-MS/MS) method for the simultaneous detection of three specific biomarkers (methylmalonic acid, methylcitric acid, and homocysteine) in DBS, as well as to appraise the applicability of these three DBS metabolites in monitoring patients with MMA, PA, and homocysteinemia during follow-up.

Methods: A total of 140 healthy controls and 228 participants were enrolled, including 205 patients with MMA, 17 patients with PA, and 6 patients with homocysteinemia.

Targeting the TRIM14/USP14 Axis Enhances Immunotherapy Efficacy by Inducing Autophagic Degradation of PD-L1.

Immunotherapy has greatly improved cancer treatment in recent years by harnessing the immune system to target cancer cells. The first immunotherapeutic agent approved by the FDA was IFNα. Treatment with IFNα can lead to effective immune activation and attenuate tumor immune evasion, but persistent treatment has been shown to elicit immunosuppressive effects.

Clinical features and ALDH5A1 gene findings in 13 Chinese cases with succinic semialdehyde dehydrogenase deficiency.

Background And Aims: To investigate the clinical features, ALDH5A1 gene variations, treatment, and prognosis of patients with succinic semialdehyde dehydrogenase (SSADH) deficiency.

Materials And Methods: This retrospective study evaluated the findings in 13 Chinese patients with SSADH deficiency admitted to the Pediatric Department of Peking University First Hospital from September 2013 to September 2023.

Results: Thirteen patients (seven male and six female patients; two sibling sisters) had the symptoms aged from 1 month to 1 year.

USP18 Antagonizes Pyroptosis by Facilitating Selective Autophagic Degradation of Gasdermin D.

As a key executioner of pyroptosis, Gasdermin D (GSDMD) plays a crucial role in host defense and emerges as an essential therapeutic target in the treatment of inflammatory diseases. So far, the understanding of the mechanisms that regulate the protein level of GSDMD to prevent detrimental effects and maintain homeostasis is currently limited. Here, we unveil that ubiquitin-specific peptidase 18 (USP18) works as a negative regulator of pyroptosis by targeting GSDMD for degradation and preventing excessive innate immune responses.

Effect of terahertz waves on the aggregation behavior of neurotransmitters.

Understanding the dynamics of neurotransmitters is crucial for unraveling synaptic transmission mechanisms in neuroscience. In this study, we investigated the impact of terahertz (THz) waves on the aggregation of four common neurotransmitters through all-atom molecular dynamics (MD) simulations. The simulations revealed enhanced nicotine (NCT) aggregation under 11.

Dual rare genetic diseases in five pediatric patients: insights from next-generation diagnostic methods.

Background: Clinicians traditionally aim to identify a singular explanation for the clinical presentation of a patient; however, in some cases, the diagnosis may remain elusive or fail to comprehensively explain the clinical findings. In recent years, advancements in next-generation sequencing, including whole-exome sequencing, have led to the incidental identification of dual diagnoses in patients. Herein we present the cases of five pediatric patients diagnosed with dual rare genetic diseases.

Detection of Cadmium(II) in Aquatic Products Using a Rolling-Circle Amplification-Coupled Ratio Fluorescent Probe Based on an Aptamer-Peptide Conjugate.

The existing aptamers for cadmium (Cd), the common toxic heavy metal contaminant in food, cannot meet the requirements for detecting Cd in rapid detection methods. In previous work, we found that coupling aptamer-peptide conjugates (APCs) with peptides and aptamers can provide a less disruptive method with a significantly improved affinity. Moreover, we found that the spatial conformation of aptamers and peptides is crucial for obtaining proper affinity in APC.

Tracking the transformation pathway of dissolved organic matters (DOMs) in biochars under sludge pyrolysis via reactomics and molecular network analysis.

This work examined the transformation pathways of sludge biochar-derived dissolved organic matters (SBC-derived DOMs) under sludge pyrolysis via FT-ICR-MS-based reactomics and molecular network analysis. Lignin/carboxylic-rich alicyclic molecules, proteins/aliphatic, and lipids of SBC-derived DOMs did not contribute equally to the overall pyrolytic reactions. Reactomics suggested that the pyrolysis reactions of SBC-derived DOMs consist of multiple cascade reactions involving the elimination of assemblages of reactive fragments during each pyrolysis reaction region, and the overall pyrolysis process was divided into three stages according to cascade reaction variations.

Case report: An asymptomatic mother with an inborn error of cobalamin metabolism (cblC) detected through high homocysteine levels during prenatal diagnosis.

Background: The most common disorder of the intracellular cobalamin metabolism pathway is the combined methylmalonic acidemia and homocysteinemia, cblC type (cblC). There is a variation in its clinical spectrum ranging from severe neonatal-onset forms that are highly fatal to later-onset forms which are milder. In this study, the first case of an asymptomatic Chinese woman with a defect in congenital cobalamin (cblC type) metabolism at prenatal diagnosis due to elevated homocysteine level is identified.

A novel fluorescent biosensor based on affinity-enhanced aptamer-peptide conjugate for sensitive detection of lead(II) in aquatic products.

Lead contamination is a major concern in food safety and, as such, many lead detection methods have been developed, especially aptamer-based biosensors. However, the sensitivity and environmental tolerance of these sensors require improvement. A combination of different types of recognition elements is an effective way to improve the detection sensitivity and environmental tolerance of biosensors.

The Protective Effect of the Extracts on Hypertensive Nephropathy Rats.

Background: Hypertensive nephropathy (HN) is one kind of kidney disorders caused by long-term uncontrolled hypertension, usually resulting in severe kidney damage, including inflammation and oxidative stress, no matter in cells or tissues, from patients with nephropathy. In recent years, nephropathy accompanied by hypertension is becoming one of the main causes for kidney replacement therapy, but few effective treatments have been reported for HN treatment. (AC) is a kind of plant with the effects of anti-inflammation, lowering blood pressure, and anti-oxidative stress.

Metabolic enzyme UAP1 mediates IRF3 pyrophosphorylation to facilitate innate immune response.

Post-translational modifications (PTMs) of proteins are crucial to guarantee the proper biological functions in immune responses. Although protein phosphorylation has been extensively studied, our current knowledge of protein pyrophosphorylation, which occurs based on phosphorylation, is very limited. Protein pyrophosphorylation is originally considered to be a non-enzymatic process, and its function in immune signaling is unknown.

Retraction: MicroRNA-433 inhibits oral squamous cell carcinoma cells by targeting FAK.

[This retracts the article DOI: 10.18632/oncotarget.22151.

Retraction Notice to: Bone Marrow Mesenchymal Stem Cell-Derived Exosomal MicroRNA-126-3p Inhibits Pancreatic Cancer Development by Targeting ADAM9.

[This retracts the article DOI: 10.1016/j.omtn.

Late-onset cblC deficiency around puberty: a retrospective study of the clinical characteristics, diagnosis, and treatment.

Background: cblC deficiency is the most common type of methylmalonic aciduria in China. Late-onset patients present with various non-specific symptoms and are usually misdiagnosed. The purpose of this study is to investigate the clinical features of patients with late-onset cblC deficiency and explore diagnosis and management strategies around puberty.

Fluorescence Resonance Energy Transfer Aptasensor of Ochratoxin A Constructed Based on Gold Nanorods and DNA Tetrahedrons.

Ochratoxin A (OTA) contamination of corn has received significant attention due to the wide distribution and high toxicity of OTA. The maximum residue limit standard of OTA in corn has been established by the Chinese Government and other unions. Nanoparticle-based fluorescence resonance energy transfer (FRET) assays are promising methods for the sensitive and fast detection of OTA.