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21.
Heidrich H. Von Helmolt C.H. Hoffmann D. Hensel H.-J. Kleinwàchter A. 《Electronics letters》1987,23(7):335-336
Experimental results for an integrated-optical state-of-polarisation (SOP) controller device on Ti:LiNbO3 for continuous broadband optical operation, with small temperature sensitivity and the possibility of integration with polarisation- insensitive directional couplers, are presented. 相似文献
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Jan H. Dring Julian Schrter Jerome Jüngling Saskia Biskup Kerstin A. Klotz Thomas Bast Tobias Dietel G. Christoph Korenke Sophie Christoph Heiko Brennenstuhl Guido Rubboli Rikke S. Mller Gaetan Lesca Yves Chaix Stefan Klker Georg F. Hoffmann Johannes R. Lemke Steffen Syrbe 《International journal of molecular sciences》2021,22(6)
Pathogenic variants in KCNA2, encoding for the voltage-gated potassium channel Kv1.2, have been identified as the cause for an evolving spectrum of neurological disorders. Affected individuals show early-onset developmental and epileptic encephalopathy, intellectual disability, and movement disorders resulting from cerebellar dysfunction. In addition, individuals with a milder course of epilepsy, complicated hereditary spastic paraplegia, and episodic ataxia have been reported. By analyzing phenotypic, functional, and genetic data from published reports and novel cases, we refine and further delineate phenotypic as well as functional subgroups of KCNA2-associated disorders. Carriers of variants, leading to complex and mixed channel dysfunction that are associated with a gain- and loss-of-potassium conductance, more often show early developmental abnormalities and an earlier onset of epilepsy compared to individuals with variants resulting in loss- or gain-of-function. We describe seven additional individuals harboring three known and the novel KCNA2 variants p.(Pro407Ala) and p.(Tyr417Cys). The location of variants reported here highlights the importance of the proline(405)–valine(406)–proline(407) (PVP) motif in transmembrane domain S6 as a mutational hotspot. A novel case of self-limited infantile seizures suggests a continuous clinical spectrum of KCNA2-related disorders. Our study provides further insights into the clinical spectrum, genotype–phenotype correlation, variability, and predicted functional impact of KCNA2 variants. 相似文献
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Anna Laitakari Lingzhi Liu Thomas M. Frimurer Birgitte Holst 《International journal of molecular sciences》2021,22(8)
The G-protein coupled receptor GPR39 is abundantly expressed in various tissues and can be activated by changes in extracellular Zn2+ in physiological concentrations. Previously, genetically modified rodent models have been able to shed some light on the physiological functions of GPR39, and more recently the utilization of novel synthetic agonists has led to the unraveling of several new functions in the variety of tissues GPR39 is expressed. Indeed, GPR39 seems to be involved in many important metabolic and endocrine functions, but also to play a part in inflammation, cardiovascular diseases, saliva secretion, bone formation, male fertility, addictive and depression disorders and cancer. These new discoveries offer opportunities for the development of novel therapeutic approaches against many diseases where efficient therapeutics are still lacking. This review focuses on Zn2+ as an endogenous ligand as well as on the novel synthetic agonists of GPR39, placing special emphasis on the recently discovered physiological functions and discusses their pharmacological potential. 相似文献
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Hui Gu Rowland M. Cannon Hans J. Seifert Michael J. Hoffmann Isao Tanaka 《Journal of the American Ceramic Society》2002,85(1):25-32
The nitrogen solubility in the SiO2 -rich liquid in the metastable binary SiO2 -Si3 N4 system has been determined by analytical TEM to be 1%–4% of N/(O + N) at 1973–2223 K. Analysis of the near edge structure of the electron energy loss peak indicates that nitrogen is incorporated into the silicate network rather than being present as molecular N2 . A regular solution model with a positive enthalpy of mixing for the liquid was used to match the data for the metastable solubility of N in the presence of crystalline Si3 N4 and to adjust the computed phase diagram. The solubility of Si3 N4 in fused SiO2 is far less than reported in liquid silicates also containing Al, Mg, and/or Y. Apparently, these cations act as modifiers that break anion bridges in the silicate network and, thereby, allow further incorporation of Si3 N4 without prohibitive amounts of network cross-linking. Finally, indications emerged regarding the diffuse nature of the Si3 N4 -SiO2 interface that leads to amorphous regions of higher N content. 相似文献
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Schüth F. Busch O. Hoffmann C. Johann T. Kiener C. Demuth D. Klein J. Schunk S. Strehlau W. Zech T. 《Topics in Catalysis》2002,21(1-3):55-66
High-throughput experimentation in catalysis comprises the following components: (i) automated high-throughput synthesis, (ii) testing in Stage I and Stage II, for which to some extent novel assays are necessary, (iii) data handling and experimental design tools, and (iv) robotics. This contribution covers these topics, using examples from the research of the authors, but also from the literature, in order to illustrate the problems and opportunities associated with high-throughput experimentation in catalysis, focusing particularly on heterogeneous catalysis. 相似文献