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排序方式: 共有162条查询结果,搜索用时 31 毫秒
1.
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
2.
In situ esterifications of high-acidity rice bran oil with methanol and ethanol and with sulfuric acid as catalyst were investigated.
In the esterification with methanol, all free fatty acids (FFA) dissolved in methanol were interesterified within 15 min,
and it was possible to obtain nearly pure methyl esters. The amount of methyl esters obtained from a given rice bran was dependent
on the FFA content of the rice bran oil. In the esterification with ethanol, it was not possible to obtain pure esters as
in methanol esterification, because the solubilities of oil components in ethanol were much higher than those in methanol. 相似文献
3.
Selma Djelloul 《Theoretical computer science》2009,410(8-10):696-710
4.
Vicente M. Gómez-López María I. Gil Ana Allende Jeroen Blancke Lien Schouteten María V. Selma 《Food and Bioprocess Technology》2014,7(12):3390-3397
The fresh-cut industry must treat process water to guarantee its microbial quality before reuse or recirculation back into the processing line. In the present study, the suitability of high-power ultrasound (HPU) for disinfecting and recycling process water was evaluated. An ultrasonic horn (20 kHz) was used to inactivate Escherichia coli O157:H7 inoculated in five types of process water which showed different physical and chemical characteristics. Differences in the inactivation level of E. coli O157:H7 at different HPU densities (0.14, 0.28, 0.56, and 1.12 kW/L) with controlled (20–25 °C) and uncontrolled (15–72 °C, 3.6 °C/min) temperature increase were studied. Results showed that the higher the power density and temperature, the higher the efficiency, reaching up to 6 log reductions of E. coli O157:H7. Alkalinity (between 0 and 253 mg HCO3 ?/L) and organic matter concentration (between 9 and 3,525 mg O2/L) in water did not reduce ultrasonic efficacy against E. coli O157:H7. Agglomerates >90 μm, which represented 34 % of those present in the process water, were reduced to only 11 % by HPU. Results indicate that HPU can be successfully applied to treat process water of the fresh produce industry because the antimicrobial efficacy was not affected by the continuous variation of the process water quality. HPU can be a suitable technology for the fresh produce industry to be able to reduce consumption of water and decrease wastewater and the generation of disinfection by-products. 相似文献
5.
Rheological,Thermodynamic, and Gas Solubility Properties of Phenylacetic Acid‐Based Deep Eutectic Solvents 下载免费PDF全文
Tausif Altamash Mert Atilhan Amal Aliyan Ruh Ullah Mustafa Nasser Santiago Aparicio 《化学工程与技术》2017,40(4):778-790
Choline chloride + phenylacetic acid‐based deep eutectic solvents are studied. Their most relevant experimental physicochemical properties at different mixing ratios together with the CO2 solubility data obtained in wide pressure and temperature ranges are reported. The presented materials exhibit a significant CO2 capture performance with low corrosion effect when compared with the most common amine‐based CO2 capture agents. Detailed rheological measurements are carried out and various models are applied to describe the dynamic flow behavior of the solvents. The CO2 absorption mechanism is evaluated by studying the behavior of the liquid gas and interface. Due to the advantages of low cost, nontoxicity, and favorable physical properties, these solvents are an environmentally promising alternative for effective CO2 capture technological applications. 相似文献
6.
Selma Ben Saad Caroline Gentric Jean-François Fourmigué Patrice Clément Jean-Pierre Leclerc 《Chemical Engineering Research and Design》2014
High performance of compact heat exchangers is conditioned by correct fluid distribution. This is especially true for gas–liquid heat exchangers where a uniform distribution is particularly delicate to obtain and where maldistribution entails significant performance deterioration. Several phenomena can lead to phase distribution problems: the fins may be subject to manufacturing defects or fouling, leading to shortcuts or dead zones. But the first source of maldistribution may be a poor distribution at the outlet of the entrance distributor. This distributor aims at mixing the phases and distributing them across the channels. 相似文献
7.
8.
Coordination‐Assisted Bioorthogonal Chemistry: Orthogonal Tetrazine Ligation with Vinylboronic Acid and a Strained Alkene 下载免费PDF全文
Selma Eising Dr. Bo‐Tao Xin Fleur Kleinpenning Jurriaan J. A. Heming Dr. Bogdan I. Florea Prof. Dr. Herman S. Overkleeft Dr. Kimberly M. Bonger 《Chembiochem : a European journal of chemical biology》2018,19(15):1648-1652
Bioorthogonal chemistry can be used for the selective modification of biomolecules without interfering with any other functionality that might be present. Recent developments in the field include orthogonal bioorthogonal reactions to modify multiple biomolecules simultaneously. During our research, we observed that the reaction rates for the bioorthogonal inverse‐electron‐demand Diels–Alder (iEDDA) reactions between nonstrained vinylboronic acids (VBAs) and dipyridyl‐s‐tetrazines were exceptionally higher than those between VBAs and tetrazines bearing a methyl or phenyl substituent. As VBAs are mild Lewis acids, we hypothesised that coordination of the pyridyl nitrogen atom to the boronic acid promoted tetrazine ligation. Herein, we explore the molecular basis and scope of VBA–tetrazine ligation in more detail and benefit from its unique reactivity in the simultaneous orthogonal tetrazine labelling of two proteins modified with VBA and norbornene, a widely used strained alkene. We further show that the two orthogonal iEDDA reactions can be performed in living cells by labelling the proteasome by using a nonselective probe equipped with a VBA and a subunit‐selective VBA bearing a norbornene moiety. 相似文献
9.
Hynek Biederman Ismail H. Boyaci Petra Bilkova Danka Slavinska Selma Mutlu Josef Zemek Miroslava Trchova Josef Klimovic Mehmet Mutlu 《应用聚合物科学杂志》2001,81(6):1341-1352
Cellulose acetate membranes (CA) were modified by means of plasma polymerization of ethylene diamine (EDA) and n‐butylamine (n‐BA). The motivation for this work was the application of a modified membrane for the single‐layer enzyme electrode. A tubular reactor with the external radiofrequency (13.56 MHz) excitation was used. Surface modification was performed at 5, 10, and 15 W power (at 27 Pa working pressure) for 5, 10, 15 min. Modified surfaces were characterized in detail by FTIR–ATR, XPS (ESCA), contact angle, and enzyme immobilization activity. The best treatment results were obtained for EDA with 5 W and 30 min and 15 W and 10 min. These results are discussed using surface analysis data. © 2001 John Wiley & Sons, Inc. J Appl Polym Sci 81: 1341–1352, 2001 相似文献
10.
Hernandez Giovanni; Hamdani Selma; Rajabi Heshmat; Conover Kent; Stewart Jane; Arvanitogiannis Andreas; Shizgal Peter 《Canadian Metallurgical Quarterly》2006,120(4):888
Extracellular dopamine levels were measured in the rat nucleus accumbens by means of in vivo microdialysis. Delivery of rewarding medial forebrain bundle stimulation at a low rate (5 trains/min) produced a sustained elevation of dopamine levels, regardless of whether train onset was predictable. When the rate of train delivery was increased to 40 trains/min, dopamine levels rose rapidly during the first 40 min but then declined toward the baseline range. The rewarding impact of the stimulation was reduced following prior delivery of stimulation at the high, but not the low, rate. These results support the idea that dopamine tone plays an enabling role in brain stimulation reward and is elevated similarly by predictable and unpredictable stimulation. (PsycINFO Database Record (c) 2010 APA, all rights reserved) 相似文献