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71.
72.
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
73.
Pharaoh Fellow Mwale Chi-Hsin Lee Peng-Nien Huang Sung-Nien Tseng Shin-Ru Shih Hsin-Yuan Huang Sy-Jye Leu Yun-Ju Huang Liao-Chun Chiang Yan-Chiao Mao Wei-Chu Wang Yi-Yuan Yang 《International journal of molecular sciences》2021,22(8)
Coxsackievirus A16 (CA16) is one of the major causative agents of hand, foot, and mouth disease (HFMD). Children aged <5 years are the most affected by CA16 HFMD globally. Although clinical symptoms of CA16 infections are usually mild, severe complications, such as aseptic meningitis or even death, have been recorded. Currently, no vaccine or antiviral therapy for CA16 infection exists. Single-chain variable fragment (scFv) antibodies significantly inhibit viral infection and could be a potential treatment for controlling the infection. In this study, scFv phage display libraries were constructed from splenocytes of a laying hen immunized with CA16-infected lysate. The pComb3X vector containing the scFv genes was introduced into ER2738 Escherichia coli and rescued by helper phages to express scFv molecules. After screening with five cycles of bio-panning, an effective scFv antibody showing favorable binding activity to proteins in CA16-infected lysate on ELISA plates was selected. Importantly, the selected scFv clone showed a neutralizing capability against the CA16 virus and cross-reacted with viral proteins in EV71-infected lysate. Intriguingly, polyclonal IgY antibody not only showed binding specificity against proteins in CA16-infected lysate but also showed significant neutralization activities. Nevertheless, IgY-binding protein did not cross-react with proteins in EV71-infected lysate. These results suggest that the IgY- and scFv-binding protein antibodies provide protection against CA16 viral infection in in vitro assays and may be potential candidates for treating CA16 infection in vulnerable young children. 相似文献
74.
Shiuh-Tarng Chiang Ding-I Liu An-Chen Lee Wei-Hua Chieng 《International Journal of Machine Tools and Manufacture》1995,35(4)
In this paper, we propose an architecture with two different kinds of neural networks for on-line determination of optimal cutting conditions. A back-propagation network with three inputs and four outputs is used to model the cutting process. A second network, which parallelizes the augmented Lagrange multiplier algorithm, determines the corresponding optimal cutting parameters by maximizing the material removal rate according to appropriate operating constraints. Due to its parallelism, this architecture can greatly reduce processing time and make real-time control possible. Numerical simulations and a series of experiments are conducted on end milling to confirm the feasibility of this architecture. 相似文献
75.
76.
Tsan-Yue Yang George E. Klinzing Shiao-Hung Chiang Fernando J. Aguirre 《Chemical Engineering Communications》1988,64(1):1-11
In the study of mass transfer between partially miscible liquids, temperature perturbations have been found to have an important effect in the generation of interfacial activity. Micro- and macro-generated interfacial convection usually occurs in combination with one another even in fairly simple heat or mass transfer events. These combinations are responsible for the enhancement of mass transfer rates. In an effort to acquire a better understanding of the heat transfer effects in ternary liquid-liquid systems, temperature difference profiles were measured when contacting two partially miscible phases. A vertical and a rotational transfer cell were designed to contact the partially miscible phases without inducing external disturbances. Five thermocouples were immersed in the bottom liquid phase at predetermined positions below the interface. In order to understand the influence of the convection generated, the vertical cell was designed to hinder convection in one phase, while the rotational cell permits convection in both phases. The experimental results showed larger temperature differences in the rotational cell after the contact of the phases. Also, systems which were initially unsaturated presented larger heat effects than those in which the partially miscible solvents were initially saturated. Several parameters indicating the importance of heat effects in the ternary systems studied are reported. 相似文献
77.
Two- and three-component polypropylene (PP) blends and composites were prepared to study their structure/property relationships. Butadiene-styrene (BDS) copolymers of low compatibility formed large particles of poor adhesion in PP resulting in inferior mechanical properties. Better miscibility of ethylene-propylene-diene (EPDM) elastomer results in more finely dispersed particles and the experimental results indicate a transition to an interpenetrating network (IPN)– like structure with increasing elastomer content. Effective impact modification can be achieved only with EPDM elastomers of sufficient miscibility. Mutual wettability and adhesion of the components determine the structure and properties in PP/elastomer/filler systems. Modification of PP by acrylic acid grafting promotes PP/filler adhesion which, in turn, results in the separate dispersion of the components. In an unmodified PP matrix, a significant amount of the filler is encapsulated by the elastomer. At low filler content, better low temperature impact strength is achieved in the case of separately dispersed components, while encapsulation is more advantageous at high filling grades. 相似文献
78.
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
79.
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
80.
Interaction‐Feature Enhanced Multiuser Model Learning for a Home Environment Using Ambient Sensors 下载免费PDF全文
Activity recognition (AR) is a key enabler for a context‐aware smart home since knowing what the residents’ current activities helps a smart home provide more desirable services. This is why AR is often used in assistive technologies for cognitively impaired people to evaluate their abilities to undertake activities of daily living. In a real‐life scenario, multiple‐resident AR has been considered as a very challenging problem, primarily due to the complexity of data association. In addition, most prior research has not considered the potential interpersonal interactions among residents to simplify complexity, especially in an environment monitored by ambient sensors. In this study, we propose two types of multiuser activity models, both of which are derived from an interaction‐feature enhanced multiuser model learning framework. These two models consider interpersonal interactions and data association for multiuser AR using ambient sensors. We then compare their performance with the other two baseline models with or without consideration of data association and interpersonal interactions. The experimental results show that the derived models outperform other baseline classifiers. Therefore, the proposed approach can increase the opportunities for providing context‐aware services for a multiresident smart home. 相似文献