72,964 results match your criteria Glycogen Storage Diseases Types I-VII


The influence of stochastic temperature fluctuations in shaping the physiological performance of the California mussel, Mytilus californianus.

J Exp Biol 2022 Jun 24. Epub 2022 Jun 24.

Department of Animal Science, University of California Davis, Davis, CA USA.

Climate change is forecasted to increase temperature variability and stochasticity. Most of our understanding of thermal physiology of intertidal organisms has come from laboratory experiments that acclimate organisms to submerged conditions and steady-state increases in temperatures. For organisms experiencing the ebb and flow of tides with unpredictable low tide aerial temperatures, the reliability of reported tolerances and thus predicted responses to climate change requires incorporation of environmental complexity into empirical studies. Read More

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GSK3B Overexpression Alleviates Posttraumatic Osteoarthritis in Mice by Promoting DNMT1-Mediated Hypermethylation of NR4A3 Promoter.

Dis Markers 2022 14;2022:4185489. Epub 2022 Jun 14.

Department of General Surgery, 1st Affiliated Hospital of Jin Zhou Medical University, Jinzhou, 121000, China.

Background: Glycogen synthase kinase 3 (GSK3B) is reported to be a protective factor for the degradation of chondrocytes by extracellular mechanisms. Nuclear receptor subfamily 4 group A member 3 (NR4A3) is a proinflammatory factor in osteoarthritis. Their regulation mechanism in posttraumatic osteoarthritis (PTOA) is not fully understood. Read More

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Comparative study on physicochemical characteristics, α-glucosidase inhibitory effect, and hypoglycemic activity of pectins from normal and Huanglongbing-infected navel orange peels.

J Food Biochem 2022 Jun 23:e14280. Epub 2022 Jun 23.

School of Public Health and Health Management, Gannan Medical University, Ganzhou, China.

This study aimed at comparing the physicochemical characteristics, α-glucosidase inhibitory effect, and hypoglycemic activity of pectins (N-NOP and H-NOP) from peels of normal and Huanglongbing (HLB)-infected Navel oranges. Results indicated the pectins were high methoxy pectins mainly composed of homogalacturonan and rhamnogalacturonan-I. The pectins exhibited similar functional groups, surface morphology, and particle size, and had no triple-helical conformation in solution. Read More

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Microencapsulated bioactive peptides from brewer's spent grain promotes antihypertensive and antidiabetogenic effects on a hypertensive and insulin-resistant rat model.

J Food Biochem 2022 Jun 23:e14283. Epub 2022 Jun 23.

Instituto de Tecnología de Alimentos, CONICET, FIQ - UNL, Santa Fe, Argentina.

The effects of microcapsules containing brewer's spent grain (BSG) peptides were evaluated on a hypertensive/insulin-resistant rat model induced by a sucrose-rich diet (SRD) administration. Animals received for 100 days the control diet (CD), SRD, and CD and SRD diets supplemented with microencapsulated peptides (CD-P and SRD-P). During the experimental period, blood pressure was monitored. Read More

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Flavivirus NS1 Triggers Tissue-Specific Disassembly of Intercellular Junctions Leading to Barrier Dysfunction and Vascular Leak in a GSK-3β-Dependent Manner.

Pathogens 2022 May 24;11(6). Epub 2022 May 24.

Division of Infectious Diseases and Vaccinology, School of Public Health, University of California, Berkeley, CA 94720-3370, USA.

The flavivirus nonstructural protein 1 (NS1) is secreted from infected cells and contributes to endothelial barrier dysfunction and vascular leak in a tissue-dependent manner. This phenomenon occurs in part via disruption of the endothelial glycocalyx layer (EGL) lining the endothelium. Additionally, we and others have shown that soluble DENV NS1 induces disassembly of intercellular junctions (IJCs), a group of cellular proteins critical for maintaining endothelial homeostasis and regulating vascular permeability; however, the specific mechanisms by which NS1 mediates IJC disruption remain unclear. Read More

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Probiotic MCC1274 Mitigates Alzheimer's Disease-Related Pathologies in Wild-Type Mice.

Nutrients 2022 Jun 19;14(12). Epub 2022 Jun 19.

Department of Biochemistry, Graduate School of Medical Sciences, Nagoya City University, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Aichi, Nagoya 467-8601, Japan.

Probiotics improve brain function, including memory and cognition, via the microbiome-gut-brain axis. Oral administration of MCC1274 ( MCC1274) improves cognitive function in mice and mild cognitive impairment (MCI) subjects, and mitigates Alzheimer's disease (AD)-like pathologies. However, its effects on wild-type (WT) mice have not yet been explored. Read More

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Discovery of GSK3β Inhibitors through In Silico Prediction-and-Experiment Cycling Strategy, and Biological Evaluation.

Molecules 2022 Jun 14;27(12). Epub 2022 Jun 14.

Therapeutics & Biotechnology Division, Korea Research Institute of Chemical Technology, 141 Gajeong-ro, Yuseong-gu, Daejeon 34114, Korea.

Direct inhibitors of glycogen synthase kinase 3β (GSK3β) have been investigated and reported for the past 20 years. In the search for novel scaffold inhibitors, 3000 compounds were selected through structure-based virtual screening (SBVS), and then high-throughput enzyme screening was performed. Among the active hit compounds, pyrazolo [1,5-a]pyrimidin-7-amine derivatives showed strong inhibitory potencies on the GSK3β enzyme and markedly activated Wnt signaling. Read More

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1,5-Benzothiazepine Derivatives: Green Synthesis, In Silico and In Vitro Evaluation as Anticancer Agents.

Molecules 2022 Jun 10;27(12). Epub 2022 Jun 10.

Department of Pharmaceutics, College of Pharmacy, Prince Sattam Bin Abdulaziz University, Al-Alkharj 11942, Saudi Arabia.

Considering the importance of benzothiazepine pharmacophore, an attempt was carried out to synthesize novel 1,5-benzothiazepine derivatives using polyethylene glycol-400 (PEG-400)-mediated pathways. Initially, different chalcones were synthesized and then subjected to a cyclization step with benzothiazepine in the presence of bleaching clay and PEG-400. PEG-400-mediated synthesis resulted in a yield of more than 95% in less than an hour of reaction time. Read More

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IKKγ/NEMO Localization into Multivesicular Bodies.

Int J Mol Sci 2022 Jun 17;23(12). Epub 2022 Jun 17.

Department of Internal Medicine, Division of Cardiology and Angiology, Otto-von-Guericke University, 39120 Magdeburg, Germany.

The NF-κB pathway is central pathway for inflammatory and immune responses, and IKKγ/NEMO is essential for NF-κB activation. In a previous report, we identified the role of glycogen synthase kinase-3β (GSK-3β) in NF-κB activation by regulating IKKγ/NEMO. Here, we show that NEMO phosphorylation by GSK-3β leads to NEMO localization into multivesicular bodies (MVBs). Read More

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Estradiol (E2) Improves Glucose-Stimulated Insulin Secretion and Stabilizes GDM Progression in a Prediabetic Mouse Model.

Int J Mol Sci 2022 Jun 15;23(12). Epub 2022 Jun 15.

Institute of Pharmacology, Toxicology and Clinical Pharmacy, Technische Universität Braunschweig, Mendelssohnstraße 1, D-38106 Braunschweig, Germany.

Female New Zealand obese (NZO) mice are an established model of preconceptional (pc.) prediabetes that progresses as gestational diabetes mellitus (GDM) during gestation. It is known that NZO mice show improvement in insulin sensitivity and glucose-stimulated insulin secretion (GSIS) during gestation in vivo. Read More

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Role of Serotonin (5-HT) in GDM Prediction Considering Islet and Liver Interplay in Prediabetic Mice during Gestation.

Int J Mol Sci 2022 Jun 9;23(12). Epub 2022 Jun 9.

Institute of Pharmacology, Toxicology and Clinical Pharmacy, Technische Universität Braunschweig, Mendelssohnstraße 1, D-38106 Braunschweig, Germany.

Gestational diabetes (GDM) is characterized by a glucose tolerance disorder. This may first appear during pregnancy or pre-exist before conception as a form of prediabetes, but there are few data on the pathogenesis of the latter subtype. Female New Zealand obese (NZO) mice serve as a model for this subpopulation of GDM. Read More

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The Prophylactic and Multimodal Activity of Two Isatin Thiosemicarbazones against Alzheimer's Disease In Vitro.

Brain Sci 2022 Jun 19;12(6). Epub 2022 Jun 19.

Institute of Biosciences & Applications, NCSR "Demokritos", Patr. Grigoriou and Neapoleos 27, 15310 Athens, Greece.

Alzheimer's disease (AD) is a multifactorial disorder strongly involving the formation of amyloid-β (Aβ) oligomers, which subsequently aggregate into the disease characteristic insoluble amyloid plaques, in addition to oxidative stress, inflammation and increased acetylcholinesterase activity. Moreover, Aβ oligomers interfere with the expression and activity of Glycogen synthase kinase-3 (GSK3) and Protein kinase B (PKB), also known as AKT. In the present study, the potential multimodal effect of two synthetic isatin thiosemicarbazones (ITSCs), which have been previously shown to prevent Aβ aggregation was evaluated. Read More

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Hepatic Mitochondrial Dysfunction and Risk of Liver Disease in an Ovine Model of "PCOS Males".

Biomedicines 2022 May 31;10(6). Epub 2022 May 31.

School of Applied Science, Edinburgh Napier University, Edinburgh EH11 4BN, UK.

First-degree male relatives of polycystic ovary syndrome (PCOS) sufferers can develop metabolic abnormalities evidenced by elevated circulating cholesterol and triglycerides, suggestive of a male PCOS equivalent. Similarly, male sheep overexposed to excess androgens in fetal life develop dyslipidaemia in adolescence. Dyslipidaemia, altered lipid metabolism, and dysfunctional hepatic mitochondria are associated with the development of non-alcoholic liver disease (NAFLD). Read More

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Hepatic and renal toxicity following the injection of copper oxide nanoparticles (CuO NPs) in mature male Westar rats: histochemical and caspase 3 immunohistochemical reactivities.

Environ Sci Pollut Res Int 2022 Jun 23. Epub 2022 Jun 23.

Department of Human Anatomy and Embryology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.

Copper nanoparticles are widely utilized in a variety of applications, including metal catalysts, semiconductors, heat transfer fluids in machine tools, and even in antibacterial medications. Forty mature healthy Westar rats were utilized in the current investigation and grouped randomly into four groups (n = 10 rats/group). Group I (G1) was kept as a control group, but G2, G3, and G4 were intraperitoneally injected with CuO NPs with a dose (5 mg, 10 mg, 25 mg/kg body weight/day) respectively for 9 days. Read More

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Selective GSK3β Inhibition Mediates an Nrf2-Independent Anti-inflammatory Microglial Response.

Mol Neurobiol 2022 Jun 23. Epub 2022 Jun 23.

Institute of Global Health and Human Ecology, School of Sciences and Engineering, The American University in Cairo, P.O. Box: 74, Cairo, Egypt.

Glycogen synthase kinase 3 (GSK3) is associated with the proinflammatory phenotype of microglia and has been shown to act in concert with nuclear factor kappa B (NF-κB). GSK3 is also a suppressor of nuclear factor erythroid 2-related factor 2 (Nrf2), the principal regulator of redox homeostasis. Agreeing with the oxidative paradigm of aging, Nrf2 is often deregulated in parainflammatory and neurodegenerative diseases. Read More

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TCR activation directly stimulates PYGB-dependent glycogenolysis to fuel the early recall response in CD8 memory T cells.

Mol Cell 2022 Jun 17. Epub 2022 Jun 17.

Department of Biochemistry and Molecular Biology, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China; Department of Immunology & National Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences (CAMS) & Peking Union Medical College, Beijing, China; State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. Electronic address:

Glycolysis facilitates the rapid recall response of CD8 memory T (Tm) cells. However, it remains unclear whether Tm cells uptake exogenous glucose or mobilize endogenous sugar to fuel glycolysis. Here, we show that intracellular glycogen rather than extracellular glucose acts as the major carbon source for the early recall response. Read More

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Chemical Profiles and Insecticidal Potential of Essential Oils Isolated from Four Species against (F.).

Plants (Basel) 2022 Jun 14;11(12). Epub 2022 Jun 14.

Aromatic Plant Research Center, 230 N 1200 E, Suite 100, Lehi, UT 84043, USA.

Although chemical pesticides have been efficiently used to manage insect pest, their overuse has led to environmental contamination and threats to human health, enticing researchers to introduce eco-friendly and effective agents. In this study, the insecticidal effectiveness of essential oils isolated from species, including , , , and , was evaluated against the adults of . The terpenes -cymene, 1,8-cineole, linalool, α-terpineol, and carvacrol were the prominent compounds in the hydrodistilled essential oils. Read More

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NMR-Based Metabolomics Identify Metabolic Change in Spleen of Idiopathic Thrombocytopenic Purpura Patients.

Metabolites 2022 Jun 19;12(6). Epub 2022 Jun 19.

Department of General Surgery, Fujian Medical University Union Hospital, No. 29, Xinquan Road, Gulou District, Fuzhou 351001, China.

Idiopathic thrombocytopenic purpura (ITP) is a common hematological disease and the abnormal platelet destruction in the spleen is a critical pathological mechanism for ITP. However, the metabolomic change in the spleen caused by ITP is still unclear. In the present study, the metabolomic information of 18 ITP and 20 normal spleen samples were detected by using H high-resolution magic angle spinning NMR spectroscopy (H MAS NMR). Read More

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Sea Bass Essence from Improves Exercise Performance and Anti-Fatigue in Mice.

Metabolites 2022 Jun 8;12(6). Epub 2022 Jun 8.

Department of Food and Nutrition, Providence University, Taichung 43301, Taiwan.

Sea bass () is rich in protein, amino acids, and long-chain omega 3 (omega-3), which have many health benefits. In East Asian food culture, soup is often eaten as a nutritional supplement. The purpose of this study was to investigate the benefits of Hi-Q sea bass essence (SBE) supplementation for improved exercise performance and anti-fatigue. Read More

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Taurine Ameliorates Streptozotocin-Induced Diabetes by Modulating Hepatic Glucose Metabolism and Oxidative Stress in Mice.

Metabolites 2022 Jun 6;12(6). Epub 2022 Jun 6.

Department of Bioscience and Biotechnology, Fukui Prefectural University, 4-1-1 Matsuoka Kenjyojima, Eiheiji 910-1195, Fukui, Japan.

Taurine is a sulfated amino acid derivative that plays an important role in maintaining the cell function of the living body. Although taurine has been shown to ameliorate diabetes, its mechanism of action has not yet been fully elucidated. The present study investigated the effects of taurine on diabetes focusing on glucose metabolism and oxidative stress. Read More

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Energy Metabolism in Mouse Sciatic Nerve A Fibres during Increased Energy Demand.

Metabolites 2022 May 31;12(6). Epub 2022 May 31.

School of Life Sciences, University of Nottingham, Nottingham NG7 2UH, UK.

The ability of sciatic nerve A fibres to conduct action potentials relies on an adequate supply of energy substrate, usually glucose, to maintain necessary ion gradients. Under our ex vivo experimental conditions, the absence of exogenously applied glucose triggers Schwann cell glycogen metabolism to lactate, which is transported to axons to fuel metabolism, with loss of the compound action potential (CAP) signalling glycogen exhaustion. The CAP failure is accelerated if tissue energy demand is increased by high-frequency stimulation (HFS) or by blocking lactate uptake into axons using cinnemate (CIN). Read More

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Promising Insecticidal Efficiency of Essential Oils Isolated from Four Cultivated Species in Iran against the Lesser Grain Borer, (F.).

Insects 2022 May 31;13(6). Epub 2022 May 31.

Department of Pre-Clinic and Applied Animal Science, Faculty of Veterinary Science, Mahidol University, Nakhon Pathom 73170, Thailand.

The lesser grain borer, (F.), causes damage to stored grains resulting in both quantitative and qualitative losses. The use of synthetic fumigants in the management of stored-product pests resulted in undesirable side effects such as environmental contamination and threat to human and animal health. Read More

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Sanghuang Tongxie Formula Ameliorates Insulin Resistance in Through Regulating PI3K/Akt Signaling.

Front Pharmacol 2022 6;13:874180. Epub 2022 Jun 6.

Hebei Key Laboratory of Integrated Traditional Chinese and Western Medicine for Diabetes and Its Complications, College of Traditional Chinese Medicine, North China University of Science and Technology, Tangshan, China.

Insulin resistance (IR) is a pivotal pathological characteristic that affects the occurrence and development of type 2 diabetes mellitus (T2DM). Thus, the effective control of IR is of great significance for diabetes prevention and treatment. Traditional Chinese medicine (TCM) represents a valuable tool handed down to the world by the Chinese nation and has a long history of use for diabetes clinical therapy. Read More

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Age-Dependent Reduction in the Expression Levels of Genes Involved in Progressive Myoclonus Epilepsy Correlates with Increased Neuroinflammation and Seizure Susceptibility in Mouse Models.

Mol Neurobiol 2022 Jun 22. Epub 2022 Jun 22.

Department of Biological Sciences and Bioengineering, Indian Institute of Technology Kanpur, Uttar Pradesh, Kanpur, 208016, India.

Brain aging is characterized by a gradual decline in cellular homeostatic processes, thereby losing the ability to respond to physiological stress. At the anatomical level, the aged brain is characterized by degenerating neurons, proteinaceous plaques and tangles, intracellular deposition of glycogen, and elevated neuroinflammation. Intriguingly, such age-associated changes are also seen in neurodegenerative disorders suggesting that an accelerated aging process could be one of the contributory factors for the disease phenotype. Read More

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Nonalcoholic steatohepatitis and mechanisms by which it is ameliorated by activation of the CNC-bZIP transcription factor Nrf2.

Free Radic Biol Med 2022 Jun 18. Epub 2022 Jun 18.

Jacqui Wood Cancer Centre, Division of Cellular Medicine, Ninewells Hospital and Medical School, University of Dundee, Dundee, DD1 9SY, Scotland, UK. Electronic address:

Non-alcoholic steatohepatitis (NASH) represents a global health concern. It is characterised by fatty liver, hepatocyte cell death and inflammation, which are associated with lipotoxicity, endoplasmic reticulum (ER) stress, mitochondrial dysfunction, iron overload and oxidative stress. NF-E2 p45-related factor 2 (Nrf2) is a transcription factor that combats oxidative stress. Read More

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Many faces and functions of GSKIP: a temporospatial regulation view.

Cell Signal 2022 Jun 18:110391. Epub 2022 Jun 18.

Department of Biochemistry, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan; Graduate Institutes of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan; Department of Biological Sciences, National Sun Yat-Sen University, Kaohsiung 804, Taiwan; Department of Medical Research, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan; Center for Cancer Research, Kaohsiung Medical University, Kaohsiung 807, Taiwan,; Neuroscience Research Center, Kaohsiung Medical University, Kaohsiung 807, Taiwan. Electronic address:

Glycogen synthase kinase 3 (GSK3)-β (GSK3β) interaction protein (GSKIP) is one of the smallest A-kinase anchoring proteins that possesses a binding site for GSK3β. Recently, our group identified the protein kinase A (PKA)-GSKIP-GSK3β-X axis; knowledge of this axis may help us decipher the many roles of GSKIP and perhaps help explain the evolutionary reason behind the interaction between GSK3β and PKA. In this review, we highlight the critical and multifaceted role of GSKIP in facilitating PKA kinase activity and its function as a scaffolding protein in signaling pathways. Read More

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Exosomal Circular RNA hsa_circ_0046060 of Umbilical Cord Mesenchymal Stromal Cell Ameliorates Glucose Metabolism and Insulin Resistance in Gestational Diabetes Mellitus via the miR-338-3p/G6PC2 Axis.

Int J Endocrinol 2022 11;2022:9218113. Epub 2022 Jun 11.

Nanjing Maternity and Child Health Care Institute, Women's Hospital of Nanjing Medical University, Nanjing Maternity and Child Health Care Hospital, Nanjing 210004, China.

Background: Impaired glucose metabolism and insulin sensitivity have been linked to the pathogenesis of gestational diabetes mellitus (GDM). Exosomes secreted by the umbilical cord mesenchymal stromal cells (UMSCs) and circular RNAs (circRNAs) derived from exosomes have been shown to be associated with the progression of GDM-related complications.

Methods: UMSCs were isolated from umbilical cords and identified through flow cytometry. Read More

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Glycogen storage diseases with liver involvement: a literature review of GSD type 0, IV, VI, IX and XI.

Orphanet J Rare Dis 2022 06 20;17(1):241. Epub 2022 Jun 20.

Division of Metabolism, Department of Pediatric Subspecialties, Ospedale Pediatrico Bambino Gesù, IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy.

Background: Glycogen storage diseases (GSDs) with liver involvement are classified into types 0, I, III, IV, VI, IX and XI, depending on the affected enzyme. Hypoglycemia and hepatomegaly are hallmarks of disease, but muscular and renal tubular involvement, dyslipidemia and osteopenia can develop. Considering the paucity of literature available, herein we provide a narrative review of these latter forms of GSDs. Read More

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Epigoitrin alleviates lipid and glucose metabolic disorders induced by a high-fat diet.

Food Funct 2022 Jun 20. Epub 2022 Jun 20.

State Key Laboratory for Conservation and Utilization of Subtropical Agro-bioresources, College of Animal Science and Technology, Guangxi University, Nanning 530004, P.R. China.

As living standards improve, obesity has become an increasingly serious health problem. Natural extracts from a wide range of sources are non-toxic and have significant potential as drugs for the prevention and treatment of obesity. We assessed 243 natural small molecules in a HepG2 fat accumulation model and found that epigoitrin (EP) from reduced intracellular fat deposition, increased short-chain acyl CoA dehydrogenase (SCAD) activity, promoted glucose uptake and glycogen storage, increased ATP production and reduced glutathione (GSH) content, reduced reactive oxygen species (ROS), and enhanced superoxide dismutase (SOD) activity. Read More

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Impact of Modified Atmospheres on Growth and Metabolism of Meat-Spoilage Relevant spp. as Predicted by Comparative Proteomics.

Front Microbiol 2022 2;13:866629. Epub 2022 Jun 2.

Lehrstuhl für Technische Mikrobiologie, Technische Universität München, Munich, Germany.

Modified atmosphere packaging (MAP) is a common strategy to selectively prevent the growth of certain species of meat spoiling bacteria. This study aimed to determine the impact of high oxygen MAP (70% O, 30% CO, red and white meats) and oxygen-free MAP (70% N, 30% CO, also white meat and seafood) on preventing the growth of spoiling photobacteria on meat. Growth of and was monitored in a meat simulation media under different gas mixtures of nitrogen, oxygen, and carbon dioxide, and samples were taken during exponential growth for a comparative proteomic analysis. Read More

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