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Progressive Myopathy and Respiratory Failure in a 7-Year-Old Boy With m.3251A>G MT-TL1 Mutation.

We report a pediatric case of severe isolated mitochondrial myopathy because of the rare m.3251A>G variant of the MT-TL1 gene. A 7-year-old boy presented to the hospital with acute-on-chronic weakness and respiratory insufficiency. Initial laboratory tests were notable for elevated lactate, aldolase, and lactate dehydrogenase. Despite a negative autoimmune panel, he was presumed to have myositis and treated with steroids and intravenous immunoglobulin. He continued to deteriorate, eventually requiring intubation and ventilation. Muscle biopsy revealed numerous ragged red fibers, abnormal intra

Taste characterization of okara and okara-derived substrates fermented by Pleurotus ostreatus mycelium.

Given its degradative capacity, mycelium may valorize okara, an underutilized agro-industrial by-product. This study aimed to conduct solid-state fermentation of okara and mixtures (okara with sawdust or wheat bran) using Pleurotus ostreatus mycelium and explore the potential of fermented okara as an alternative food, focusing on its taste characteristics. The fastest mycelial growth with dense cultures was achieved on non-dried okara, followed by wheat bran (11-12 days). Fermentation enhanced the nitrogen content in okara, indicating an improved protein level. Following biotransformation, sub

ILC3s regulate the gut microbiota via host intestinal galactosylation to limit pathogen infection in mice.

Host immunity and commensal bacteria synergistically maintain intestinal homeostasis and mediate colonization resistance against pathogens. However, the molecular and cellular mechanisms remain unclear. Here, with a mouse infection model of Citrobacter rodentium, a natural mouse intestinal pathogen that mimics human enteropathogenic Escherichia coli and enterohaemorrhagic Escherichia coli, we find that group 3 innate lymphoid cells (ILC3s) can protect the host from infection by regulating gut microbiota. Mechanistically, ILC3s can control gut dysbiosis through IL-22-dependent regulation of int

Interactional Effects of Food Macronutrients with Gut Microbiome: Implications for Host Health and Risk.

Macronutrients in the diet (e.g., carbohydrates, proteins, and lipids) that are rarely absorbed in the small intestine can typically traverse to the colon where they are metabolized by the gut microbiota to produce a spectrum of beneficial or detrimental metabolites. These gut-microbiota-dependent small-molecule metabolites are subsequently absorbed by the intestinal mucosa to participate in various physiological responses. Notably, succinate as a star dicarboxylic acid metabolite stemming from microbial fermentation of nondigestible carbohydrates regulates blood glucose homeostasis by promoti

Emerging therapy strategies for energy metabolism in acute myocardial infarction.

Acute myocardial infarction (AMI) is a life-threatening cardiovascular event caused by the sudden blockage of a coronary artery, usually triggered by thrombotic events. This leads to significant myocardial ischaemia and hypoxia, disrupting the heart's energy metabolism and ultimately resulting in irreversible injury to cardiomyocytes. Despite advancements in reperfusion therapies, ischaemia-reperfusion injury (IRI) remains a critical contributor to complications such as arrhythmias and heart failure. This review explores the pivotal role of myocardial energy metabolism in AMI pathogenesis, foc

Диметилфумарат способствует разложению HNF1B и подавляет прогрессирование чистой почечной карциномы.

Чистая почечная карцинома (ccRCC) является самым смертельным подтипом рака почек, и его варианты лечения по-прежнему ограничены. Поэтому необходимо срочно найти лечебные агенты для лечения CCRCC. Здесь мы показываем, что диметилфумарат (ДМФ), одобренное лекарство от множественных склерозов [1] и псориаза, может сдерживать распространение клеток ЦКЦК. Механистически, гепатоцитовый ядерный фактор 1β (HNF1B), коэффициент транскрипции, ярко выраженный в CCRCC, поддерживается DMF в остатках цистейна, что ведет к его протеасомальной деградации. Кроме того, HNF1B взаимодействует с протеином, связанны

Совпадение в полном тексте источника
… Collectively, these results indicate that DMF promotes succination of HNF1B, impairing its interaction with OTUD3, which in turn enhances its ubiquitination and degradation. HNF1B succination increases its ubiquitination. … As shown in Fig. 6I , DMF succinates HNF1B, leading to its proteasomal degradation and subsequent inactivation of YAP, which inhibits cell proliferation of ccRCC. …

The role of mitochondrial damage-associated molecular patterns in acute pancreatitis.

Acute pancreatitis (AP) is one of the most common gastrointestinal tract diseases with significant morbidity and mortality. Current treatments remain unspecific and supportive due to the severity and clinical course of AP, which can fluctuate rapidly and unpredictably. Mitochondria, cellular power plant to produce energy, are involved in a variety of physiological or pathological activities in human body. There is a growing evidence indicating that mitochondria damage-associated molecular patterns (mtDAMPs) play an important role in pathogenesis and progression of AP. With the pro-inflammatory

Negatively Charged Thermosensitive Hydrogel Loaded with Pectin Microspheres to Recover the Mucosal Barrier for Ulcerative Colitis Therapy.

Ulcerative colitis (UC), a chronic inflammatory bowel disease, poses a heightened colorectal cancer risk due to persistent mucosal inflammation and barrier dysfunction. In this article, a negatively charged thermosensitive hydrogel loaded with pectin microspheres was used as the enema for UC treatment. Succinic acid was immobilized on poly(ε-caprolactone-co-glycolide)-poly(ethylene glycol)-poly(ε-caprolactone-co-glycolide) (PCLGA-PEG-PCLGA) triblock copolymers to preferentially coat on cationic-inflamed sites via electrostatic interaction for reconstructing the mucosal barrier. Anti-inflammati

Experience of treatment in critical Guillain-Barre Syndrome case after COVID-19 vaccination.

BACKGROUND: The current study reported a case with a history of neuroradiculitis. Within 2 months of the COVID-19 vaccine, critical Guillain-Barre Syndrome (GBS) appeared after acute diarrhea, progressive myasthenia, and sudden respiratory and cardiac symptoms. METHODS: The syndrome was addressed with measures, such as endotracheal intubation and cardiopulmonary resuscitation vasoactive drugs. Next, we conducted six cycles of human immunoglobulin treatment (dose of 400 mg/kg·d intravenously for 5 days consecutively) and three times plasma exchange (PE, 30 ml/kg), followed by methylprednisolon

Application of MicroResp™ for quick and easy detection of plastic degradation by marine bacterial isolates.

Microplastic debris in the marine environment is a global problem. Biodegradable polymers are being developed as alternatives to petroleum-based plastics, and quick and easy methods for screening for bacterial strains that can degrade such polymers are needed. As a screening method, the clear zone method has been widely used but has technical difficulties such as plate preparation and interpretation of results. In this study, we adapted the MicroResp™ system to easily detect biodegradation activity of marine bacteria in a 3-day assay. Among the 6 bacterial strains tested, 3, 2 and 1 strain deg

Enzymatic Degradation Behavior of Self-Degradable Lipase-Embedded Aliphatic and Aromatic Polyesters and Their Blends.

Over the past decade, the preparation of novel materials by enzyme-embedding into biopolyesters has been proposed as a straightforward method to produce self-degrading polymers. This paper reports the preparation and enzymatic degradation of extruded self-degradable films of three different biopolyesters: poly(lactic acid) (PLA), poly(butylene adipate-co-terephthalate) (PBAT), and poly(butylene succinate) (PBS), as well as three binary/ternary blends. Candida antarctica lipase B (CalB) has been employed for the enzyme-embedding procedure, and to the best of our knowledge, the use of this appro

SGLT2 Inhibitor for Cardiac Protection in a Patient With Osimertinib-Responsive Advanced EGFR-Positive Lung Cancer.

An 85-year-old man with recently diagnosed metastatic EGFR+ lung adenocarcinoma treated with osimertinib presented after 1 month of therapy in decompensated congestive heart failure along with atrial fibrillation, prolonged QTc and acute kidney injury. Osimertinib was held. His hemodynamic status was optimized, and he was started on cardioprotective medications (losartan and metoprolol succinate), and LVEF recovered. However, after reintroducing osimertinib, LVEF reduced, indicating possible osimertinib-induced cardiomyopathy. An SGLT2 inhibitor was added for cardioprotection before another re

Isopropanol production using engineered Corynebacterium glutamicum from waste rice straw biomass.

Isopropanol, a well-known biofuel, is a widely used precursor for chemical products that can replace nonrenewable petroleum energy. Here, engineered Corynebacterium glutamicum that can effectively utilize all xylose and glucose in agricultural waste rice straw to produce isopropanol was described. First, codon mutations were introduced into transporters and glycolytic-related genes to decrease the glucose preference of C. glutamicum. A more energetically favorable xylose oxidative pathway was constructed that replaced traditional xylose isomerization pathways, saving twice the number of enzyma

Identification and Quantification of Dimethachlon Degradation Products in Soils and Their Effects on Soil Enzyme Activities.

In this study, high-performance liquid chromatography-high-resolution mass spectrometry (HPLC-HRMS, Q-Exactive Orbitrap) and Compound Discoverer 3.3 were used to screen dimethachlon degradation products in soils. Four metabolites 4-(3,5-dichloroanilino)-4-oxobutanoic acid (DCBAA), 3,5-dichloroaniline (3,5-DCA), succinic acid, and muconic acid were confirmed by primary and secondary ion mass spectrometry comparisons between standards and samples. A quantitative analysis method of dimethachlon residues and four metabolites in soils was developed using HPLC-HRMS. Dimethachlon degradation in agric

Sleep, Narcolepsy, and Sodium Oxybate.

Sodium oxybate (SO) has been in use for many decades to treat narcolepsy with cataplexy. It functions as a weak GABAB agonist but also as an energy source for the brain as a result of its metabolism to succinate and as a powerful antioxidant because of its capacity to induce the formation of NADPH. Its actions at thalamic GABAB receptors can induce slow-wave activity, while its actions at GABAB receptors on monoaminergic neurons can induce or delay REM sleep. By altering the balance between monoaminergic and cholinergic neuronal activity, SO uniquely can induce and prevent cataplexy. The forma

Discovery of Novel Benzo[4,5]thiazolo(oxazolo)[3,2-a]pyrimidinone Mesoionic Derivatives as Potential Antibacterial Agents and Mechanism Research.

A series of benzo[4,5]thiazole(oxazole)[3,2-a]pyrimidine mesoionic compounds were designed and synthesized. Antibacterial activity tests revealed that compound A23 showed good in vitro activities against Xanthomonas oryzae pv. Oryzicola (Xoc) and Xanthomonas oryzae pv. oryzae (Xoo), with half-maximal effective concentration (EC50) values of 47.6 and 36.8 μM, respectively, which were better than positive control agents thiodiazole copper (281 and 259 μM) and bismerthiazol (245 and 220 μM). The protective activities of compound A23 anti-Xoc and anti-Xoo were 39.7% and 49.2%, respectively, which

Atypical primary pulmonary amyloidosis: A rare case report.

RATIONALE: Pulmonary amyloidosis is a rare respiratory disease characterized by amyloid deposition in the lungs. The clinical manifestations of pulmonary amyloidosis are variable and without specific symptoms. PATIENT CONCERNS: We report a rare case of tracheobronchial amyloidosis to improve our understanding of the disease. DIAGNOSES: The diagnosis of tracheobronchial amyloidosis was finally established by transbronchoscopic lung biopsy and histological examination. INTERVENTIONS: The patient significantly improved with methylprednisolone sodium succinate for injection (40 mg/day) for 5 da

Nickel oxide nanoparticles exert selective toxicity on skin mitochondria and lysosomes isolated from the mouse model of melanoma.

Nickel oxide nanoparticles (NiO-NPs) are progressively used for an immense number of new applications in modern industries sectors. Nevertheless, the toxic impact of NiO-NPs has not been clearly elucidated on human melanoma cell lines at the cellular and molecular level. Hence, this study was designed to examine the in vitro cytotoxicity potentials of NiO-NPs on malignant cutaneous melanoma (MCM) mitochondria. Results revealed that NiO-NPs significantly increased reactive oxygen species level, lipid peroxidation, and mitochondrial membrane potential and decreased succinate dehydrogenase activi

Nitric Oxide Modulates Metabolic Remodeling in Inflammatory Macrophages through TCA Cycle Regulation and Itaconate Accumulation.

Classical activation of macrophages (M(LPS+IFNγ)) elicits the expression of inducible nitric oxide synthase (iNOS), generating large amounts of NO and inhibiting mitochondrial respiration. Upregulation of glycolysis and a disrupted tricarboxylic acid (TCA) cycle underpin this switch to a pro-inflammatory phenotype. We show that the NOS cofactor tetrahydrobiopterin (BH4) modulates IL-1β production and key aspects of metabolic remodeling in activated murine macrophages via NO production. Using two complementary genetic models, we reveal that NO modulates levels of the essential TCA cycle metabol

Bacterial adaptability of enzyme and pH dual-responsive surface for infection resistance.

A major challenge in antibacterial surface preparation is the elaborated implementation of controlled antibacterial agent delivery on demand, typically at appropriate concentrations and sites, which can be an efficient way to lower bacteria resistance and improve therapy effectiveness. Herein, we present a bacterial hyaluronidase (HAase) and pH dual-responsive antimicrobial surface, which is constructed by sequential layer-by-layer (LbL) assembly of NHS-PEG-NHS (PEG-bis(succinimidyl succinate)) and PEI (polyethylenimine), immobilization of antibiotic vancomycin (Van) by an acid-labile β-carbox