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Ronald J.A. Wanders,Gepke Visser,Sacha Ferdinandusse,Frédéric M. Vaz,Riekelt H. Houtkooper 한국지질동맥경화학회 2020 지질·동맥경화학회지 Vol.9 No.3
Mitochondrial fatty acid (FA) oxidation deficiencies represent a genetically heterogeneous group of diseases in humans caused by defects in mitochondrial FA beta-oxidation (mFAO). A general characteristic of all mFAO disorders is hypoketotic hypoglycemia resulting from the enhanced reliance on glucose oxidation and the inability to synthesize ketone bodies from FAs. Patients with a defect in the oxidation of long-chain FAs are at risk to develop cardiac and skeletal muscle abnormalities including cardiomyopathy and arrhythmias, which may progress into early death, as well as rhabdomyolysis and exercise intolerance. The diagnosis of mFAO-deficient patients has greatly been helped by revolutionary developments in the field of tandem mass spectrometry (MS) for the analysis of acylcarnitines in blood and/or urine of candidate patients. Indeed, acylcarnitines have turned out to be excellent biomarkers; not only do they provide information whether a certain patient is affected by a mFAO deficiency, but the acylcarnitine profile itself usually immediately points to which enzyme is likely deficient. Another important aspect of acylcarnitine analysis by tandem MS is that this technique allows high-throughput analysis, which explains why screening for mFAO deficiencies has now been introduced in many newborn screening programs worldwide. In this review, we will describe the current state of knowledge about mFAO deficiencies, with particular emphasis on recent developments in the area of pathophysiology and treatment.
Carbohydrate-containing Triton X-100 analogues for membrane protein solubilization and stabilization
Chae, Pil Seok,Wander, Marc J.,Cho, Kyung Ho,Laible, Philip D.,Gellman, Samuel H. The Royal Society of Chemistry 2013 Molecular bioSystems Vol.9 No.4
<P>Membrane protein manipulation is a challenging task owing to limited tertiary and quaternary structural stability once the protein has been removed from a lipid bilayer. Such instability can be overcome by embedding membrane proteins in detergent micelles formed from amphiphiles with carefully tuned properties. This study introduces a class of easy-to-synthesize amphiphiles, which are designated CGT (Chae's Glyco-Triton) detergents. Some of the agents are well suited for membrane protein solubilization and stabilization.</P> <P>Graphic Abstract</P><P>This study introduces a class of easy-to-synthesize amphiphiles that display favorable behaviour for membrane protein solubilization and stabilization. <IMG SRC='http://pubs.rsc.org/services/images/RSCpubs.ePlatform.Service.FreeContent.ImageService.svc/ImageService/image/GA?id=c3mb25584k'> </P>
Dutt Shivam,Tandon Rohit,Sondh Mandeep Singh,Bansal Namita,Singh Gurbhej,Mohan Bishav,Singh Bhupinder,Wander Gurpreet Singh 아시아심장혈관영상의학회 2021 Cardiovascular Imaging Asia Vol.5 No.1
Objective: Documenting adverse remodeling in absence of left ventricular outflow tract (LVOT) obstruction and mitral regurgitation in a classical hypertrophic cardiomyopathy (HCMP) phenotype is difficult. Changes in the left atrium (LA) are a consequence of progressive left ventricular (LV) fibrosis and have been shown to progress in a linear fashion. Therefore, studying LA changes for identifying adverse remodeling in HCMP patients is important. Materials and Methods: This was a prospective study which included HCMP patients and age- and gender-matched controls. Various echocardiographic parameters of adverse cardiac remodeling were investigated. Results: A total of 160 patients with HCMP and 75 age- and sex-matched controls were analyzed over a 5-year period. HCMP patients had an enlarged LA, greater segmental thickness, and mildly increased LV filling pressure. Patients with maximum LA volume >40 mL and global LA strain <21.5% showed greater maximum segmental thickness and increased ratio of pulse wave Doppler derived mitral E wave and tissue Doppler derived annular e wave with reduced LA strain and LV strain, LA emptying fraction, and strain-derived LV ejection fraction. In both groups, significant difference was not observed in age, sex, HCMP phenotype, presence or absence of LVOT obstruction, LV volume, and mitral Doppler ratio of pulse wave Doppler derived mitral E and A waves. Conclusion: Monitoring HCMP in asymptomatic patients is challenging. Assessment of adverse cardiac remodeling in classical HCMP phenotype is feasible using global LA strain and maximum LA volume. Global LA strain identifies early changes and maximum LA volume late changes of cardiac remodeling and therefore provide an early indication of disease progression in asymptomatic HCMP patients.
Melina Ribeiro Fernandes,Karoline Silva Rezende,Aline Carla Inada,Karine de Cássia Freitas,Wander Fernando de Oliveira Filiú,Leandro Fontoura Cavalheiro,Carlos Eduardo Domingues Nazário,Priscila Aiko 한국식품영양과학회 2021 Journal of medicinal food Vol.24 No.8
Obesity is a relevant health hazard characterized as a chronic noncommunicable disease, with severe comorbidities that cause mortality worldwide. Acrocomia aculeata is a Brazilian palm with edible fruits. Its pulp contains fibers, monounsaturated fatty acids (MUFAs), such as oleic acid and carotenoids. In this context, our study aimed to elucidate the protective effect of the lyophilized A. aculeata pulp added at the rates of 1%, 2%, and 4% to a high-fat (HF) diet (rich in saturated fats and cholesterol), for 90 days, in mice. The treatment with 4% pulp induced a significant increase in the biochemical parameters of serum cholesterol HDL-C (high-density lipoprotein) compared with the control. According to the evaluation of the epididymal tissue, the groups treated with A. aculeata pulp exhibited smaller fat deposits compared with the HF diet group. Therefore, we infer that the predominant components in A. aculeata, particularly fibers and MUFAs, promote beneficial effects on health parameters during simultaneous exposure to food rich in saturated fat and cholesterol, typical of the Western diet. This is the first study to correlate the presence of fatty acids from A. aculeata pulp in different proportions added in a HF diet with metabolic and histological parameters in Swiss mice.
Vitoria Helena de Oliveira Teixeira Reis,Cynthia Monteiro Santee,Paulo Se´rgio Loubet Filho,Thayna´ Gil Santos,Carolina Amianti,Wander Fernando de Oliveira Filiu,Bruna Paola Murino Rafacho,Luciane Can 한국식품영양과학회 2019 Journal of medicinal food Vol.22 No.12
The prevalence of gastrointestinal disorders, such as constipation, has been increasing. Genetic factors and lifestyle are some of the etiologies of this issue, affecting the health of the population. Natural products have properties that contribute to health maintenance and health promotion, including reduction of the inflammatory process. Hancornia speciosa, popularly known as mangaba, is an abundant and native fruit in the Brazilian Cerrado, commercialized for culinary purposes and used because of its pharmacological properties. The objective of this study was to evaluate if the supplementation of different concentrations of mangaba pulp can improve intestinal motility and bowel health in Wistar rats. Forty male rats were divided into five groups. The experiment lasted 14 days and the groups were tested with water, industrialized laxative jelly made from tamarind as medication, or mangaba at 5, 10, and 15 mL/kg of body weight. Food intake, weight gain, ion balance, intestinal motility, and histopathological analysis of the small intestine, large intestine, and liver were evaluated. Supplementation of mangaba pulp at its highest concentration (15 mL/kg body weight) caused a 15% increase in the distance traveled by the charcoal meal, and a decrease in serum magnesium levels and white cells in both the small and large intestines. The results suggest that mangaba pulp presents laxative, anti-inflammatory properties and that its consumption is beneficial and should be encouraged.
Status of the 25 T, 100 mm Bore HTS Solenoid for an Axion Dark Matter Search Experiment
Gupta, Ramesh,Anerella, Michael,Cozzolino, John,Joshi, Piyush,Joshi, Shresht,Plate, Stephen,Sampson, William,Song, Honghai,Wanderer, Peter,Chung, Woohyun,Kim, Jingeun,Ko, Byeong Rok,Youn, Sung Woo,Sem Institute of Electrical and Electronics Engineers 2019 IEEE transactions on applied superconductivity Vol.29 No.5
<P>This paper presents the design and test results of the pancake coils for the 25 T, 100 mm bore solenoid that Brookhaven National Laboratory (BNL) is building for the Institute for Basic Science (IBS) in Korea for an Axion dark matter search. The design is based on second-generation (2G) high-temperature superconducting (HTS) tape with no-insulation winding. The major challenges in the high-field, large aperture solenoid are the large stresses and the quench protection. Moreover, the design should be robust for reliable operation in a user facility environment. The paper will also present the construction and test results of two ∼100 mm bore double pancake coils creating a peak field of up to ∼17 T and similar hoop stresses as will be in the 25 T solenoid. The coils were subject to several severe tests, including the simulations of large defects and extended quench studies at ∼4 K. The most striking part of these studies was the demonstration of how fast (a few hundred milliseconds) these coils can turn from the superconducting state to the normal state (quench or thermal runaway). This removes the past concerns of protecting high-field HTS coils because of the low quench propagation velocities.</P>