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51.
52.
Anna Kutschenko Selma Staege Karen Grütz Hannes Glaß Norman Kalmbach Thomas Gschwendtberger Lisa M. Henkel Johanne Heine Anne Grünewald Andreas Hermann Philip Seibler Florian Wegner 《International journal of molecular sciences》2021,22(7)
Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by mutations in the SGCE gene encoding ε-sarcoglycan leading to a dysfunction of this transmembrane protein, alterations in the cerebello-thalamic pathway and impaired striatal plasticity. To elucidate underlying pathogenic mechanisms, we investigated induced pluripotent stem cell (iPSC)-derived striatal medium spiny neurons (MSNs) from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene (c.298T>G and c.304C>T with protein changes W100G and R102X) in comparison to two matched healthy control lines. Calcium imaging showed significantly elevated basal intracellular Ca2+ content and lower frequency of spontaneous Ca2+ signals in SGCE MSNs. Blocking of voltage-gated Ca2+ channels by verapamil was less efficient in suppressing KCl-induced Ca2+ peaks of SGCE MSNs. Ca2+ amplitudes upon glycine and acetylcholine applications were increased in SGCE MSNs, but not after GABA or glutamate applications. Expression of voltage-gated Ca2+ channels and most ionotropic receptor subunits was not altered. SGCE MSNs showed significantly reduced GABAergic synaptic density. Whole-cell patch-clamp recordings displayed elevated amplitudes of miniature postsynaptic currents and action potentials in SGCE MSNs. Our data contribute to a better understanding of the pathophysiology and the development of novel therapeutic strategies for myoclonus-dystonia. 相似文献
53.
Elvira S. Sandin Julica Folberth Helge Müller-Fielitz Claus U. Pietrzik Elisabeth Herold Thomas E. Willnow Paul T. Pfluger Ruben Nogueiras Vincent Prevot Thomas Krey Markus Schwaninger 《International journal of molecular sciences》2021,22(9)
The mechanisms underlying the transport of leptin into the brain are still largely unclear. While the leptin receptor has been implicated in the transport process, recent evidence has suggested an additional role of LRP2 (megalin). To evaluate the function of LRP2 for leptin transport across the blood-brain barrier (BBB), we developed a novel leptin-luciferase fusion protein (pLG), which stimulated leptin signaling and was transported in an in vitro BBB model based on porcine endothelial cells. The LRP inhibitor RAP did not affect leptin transport, arguing against a role of LRP2. In line with this, the selective deletion of LRP2 in brain endothelial cells and epithelial cells of the choroid plexus did not influence bodyweight, body composition, food intake, or energy expenditure of mice. These findings suggest that LRP2 at the BBB is not involved in the transport of leptin into the brain, nor in the development of obesity as has previously been described. 相似文献
54.
Elisabeth M. Haberl Thomas S. Weiss Georg Peschel Kilian Weigand Nikolai Khler Josch K. Pauling Jürgen J. Wenzel Marcus Hring Sabrina Krautbauer Gerhard Liebisch Christa Buechler 《International journal of molecular sciences》2021,22(10)
Hepatocellular carcinoma (HCC) still remains a difficult to cure malignancy. In recent years, the focus has shifted to lipid metabolism for the treatment of HCC. Very little is known about hepatitis B virus (HBV) and C virus (HCV)-related hepatic lipid disturbances in non-malignant and cancer tissues. The present study showed that triacylglycerol and cholesterol concentrations were similar in tumor adjacent HBV and HCV liver, and were not induced in the HCC tissues. Higher levels of free cholesterol, polyunsaturated phospholipids and diacylglycerol species were noted in non-tumorous HBV compared to HCV liver. Moreover, polyunsaturated phospholipids and diacylglycerols, and ceramides declined in tumors of HBV infected patients. All of these lipids remained unchanged in HCV-related HCC. In HCV tumors, polyunsaturated phosphatidylinositol levels were even induced. There were no associations of these lipid classes in non-tumor tissues with hepatic inflammation and fibrosis scores. Moreover, these lipids did not correlate with tumor grade or T-stage in HCC tissues. Lipid reprogramming of the three analysed HBV/HCV related tumors mostly resembled HBV-HCC. Indeed, lipid composition of non-tumorous HCV tissue, HCV tumors, HBV tumors and HBV/HCV tumors was highly similar. The tumor suppressor protein p53 regulates lipid metabolism. The p53 and p53S392 protein levels were induced in the tumors of HBV, HCV and double infected patients, and this was significant in HBV infection. Negative correlation of tumor p53 protein with free cholesterol indicates a role of p53 in cholesterol metabolism. In summary, the current study suggests that therapeutic strategies to target lipid metabolism in chronic viral hepatitis and associated cancers have to consider disease etiology. 相似文献
55.
Thronsen Elisabeth Mørkeseth Hanne Marioara Calin D. Minakuchi Kazuhiro Katsumi Tetsuya Marthinsen Knut Matsuda Kenji Holmestad Randi 《Metallurgical and Materials Transactions A》2022,53(9):3296-3310
Metallurgical and Materials Transactions A - The effect of 0.03 and 0.08 at. pct Fe additions on the formation of secondary phases in an Al–1.1Mg–0.5Cu–0.3Si at. pct alloy was... 相似文献
56.
57.
Social medias have changed and challenge the way we interact with each other. Social medias, such as Facebook, open up new possibilities for presentation of self and of managing the self you present to others. Is this process different for those that have grown up with social medias (The Net Generation) [Tapscott (Growing up digital: the rise of the net generation. Mcgraw-Hill, NY, 1998)] from how an older group of social media users would do it? What is their primary use of Facebook and how does this differ between generations? Such questions are discussed through engaging a group of Facebook users, with clear ideas of why and how they use Facebook, in joint reflections. The participants represent two generations of internet users; Those who was introduced to internet and social medias, such as Facebook, as adults (i.e. 40 years and older) and those who have grown up with the technology (i.e. under 25 years old, also called “The Net Generation”). The discussion indicate that there are differences in how these two groups relate to social medias, such as Facebook and for what they use Facebook. Further research is necessary to pursue those differences. 相似文献
58.
Mette B. Let Charlotte Jacobsen Edwin N. Frankel Anne S. Meyer 《European Journal of Lipid Science and Technology》2003,105(9):518-528
The oxidative deterioration of milk emulsions supplemented with 1.5 wt‐% fish oil was investigated by sensory evaluation and by determining the peroxide value and volatile oxidation products after cold storage. Two types of milk emulsions were produced, one with a highly unsaturated tuna oil (38 wt‐% of n‐3 fatty acids) and one with cod liver oil (26 wt‐% of n‐3 fatty acids). The effect of added calcium disodium ethylenediaminetetraacetate (EDTA) on oxidation was also investigated. Emulsions based on cod liver oil with a slightly elevated peroxide value (1.5 meq/kg) oxidised significantly faster than the tuna oil emulsions, having a lower initial peroxide value (0.1 meq/kg). In the tuna oil emulsions the fishy off‐flavour could not be detected throughout the storage period. Addition of 5—50 ppm EDTA significantly reduced the development of volatile oxidation products in the cod liver oil emulsions, indicating that metal chelation with EDTA could inhibit the decomposition of lipid hydroperoxides in these emulsions. This study showed that an oxidatively stable milk emulsion containing highly polyunsaturated tuna fish oil could be prepared without significant fishy off‐flavour development upon storage, provided that the initial peroxide value was sufficiently low. 相似文献
59.
Lisbeth M Ottosen Ana T Lima Anne J Pedersen Alexandra B Ribeiro 《Journal of chemical technology and biotechnology (Oxford, Oxfordshire : 1986)》2006,81(4):553-559
The possibility of using fly ash from municipal solid waste incineration (MSWI) in, for example, concrete is considered. MSWI fly ash, however, has too high a concentration of heavy metals, which may cause leaching problems during use or problems with waste handling at the end of the lifetime of the concrete. The Cl content in MSWI fly ash is also too high and will cause corrosion problems in reinforced concrete. The possibility of removing some of the unwanted heavy metals (Cu and Pb) together with Cl from an MSWI fly ash suspended in water using an electrodialytic separation method was investigated. The removal of Pb and Cu was found to be highly pH dependent and the highest contents removed were 41 and 90%, respectively. The Cu concentration of the ash decreased from 2200 to 860 mg kg?1 but the Pb concentration increased from 8560 to 16 800 mg kg?1, showing that Pb is mainly found in the ash fraction that is least soluble. Hence electrodialytic treatment of the ash suspended in water is not a solution to improve the ash quality in terms of Pb. The water‐soluble Cl content per unit weight of the original ash was 12.4%. The removal of water‐soluble Cl was efficient and >98% of Cl was removed (calculated on the basis of mean initial and final concentrations). This result indicates that electrodialytic extraction may be a method that can be used for the removal of Cl from ash prior to its utilization in concrete. Copyright © 2006 Society of Chemical Industry 相似文献
60.
Dehydrogenation of Light Alkanes Over Rhenium Catalysts on Conventional and Mesoporous MFI Supports 总被引:1,自引:0,他引:1
Anne Krogh Rovik Anke Hagen Iver Schmidt Søren Dahl Ib Chorkendorff Claus Hviid Christensen 《Catalysis Letters》2006,109(3-4):153-156
Recently, Re/HZSM-5 (Si/Al = 15) was shown to be an efficient catalyst for ethane dehydrogenation and aromatization at 823 K
and atmospheric pressure. In this reaction, the major initial products were benzene, toluene and xylene (BTX), but increasing
amounts of ethene were produced with time on stream due to deactivation of the catalyst. We show that by use of rhenium impregnated
MFI supports with very few or no acidic sites (Si/Al > 500), highly selective ethane dehydrogenation catalysts are obtained
with ethene selectivities of 98%. By use of mesoporous MFI supports (Si/Al >500) the lifetime of the catalyst appears to be
slightly improved compared to conventional MFI crystals. The beneficial effect of a mesoporous MFI support is convincingly
demonstrated in propane dehydrogenation, where both conversion and selectivities on the mesoporous MFI (Si/Al > 500) impregnated
with Re are significantly higher than on Re supported on a comparable conventional MFI support. 相似文献