排序方式: 共有172条查询结果,搜索用时 15 毫秒
141.
Mutations in MDH2, Encoding a Krebs Cycle Enzyme,Cause Early-Onset Severe Encephalopathy 总被引:1,自引:0,他引:1
Samira Ait-El-Mkadem Manal Dayem-Quere Mirjana Gusic Annabelle Chaussenot Sylvie Bannwarth Bérengère François Emmanuelle C. Genin Konstantina Fragaki Catharina L.M. Volker-Touw Christelle Vasnier Valérie Serre Koen L.I. van Gassen Françoise Lespinasse Susan Richter Graeme Eisenhofer Cécile Rouzier Fanny Mochel Anne De Saint-Martin Véronique Paquis-Flucklinger 《American journal of human genetics》2017,100(1):151-159
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c-SRC mediates neurite outgrowth through recruitment of Crk to the scaffolding protein Sin/Efs without altering the kinetics of ERK activation 总被引:3,自引:0,他引:3
SRC family kinases have been consistently and recurrently implicated in neurite extension events, yet the mechanism underlying their neuritogenic role has remained elusive. We report that epidermal growth factor (EGF) can be converted from a non-neuritogenic into a neuritogenic factor through moderate activation of endogenous SRC by receptor-protein-tyrosine phosphatase alpha (a physiological SRC activator). We show that such a qualitative change in the response to EGF is not accompanied by changes in the extent or kinetics of ERK induction in response to this factor. Instead, the pathway involved relies on increased tyrosine phosphorylation of, and recruitment of Crk to, the SRC substrate Sin/Efs. The latter is a scaffolding protein structurally similar to the SRC substrate Cas, tyrosine phosphorylation of which is critical for migration in fibroblasts and epithelial cells. Expression of a dominant negative version of Sin interfered with receptor-protein-tyrosine phosphatase alpha/EGF- as well as fibroblast growth factor-induced neurite outgrowth. These observations uncouple neuritogenic signaling in PC12 cells from sustained activation of ERK kinases and for the first time identify an effector of SRC function in neurite extension. 相似文献
143.
Konstantina Skorda Anastasios P. Vafiadis Aris Terzis Jerzy Mrozinski Catherine P. Raptopoulou John C. Plakatouras Evangelos G. Bakalbassis 《Inorganica chimica acta》2005,358(3):565-582
A systematic investigation of the CuCl2/Mebta (Mebta = 1-methylbenzotriazole) reaction system is described, involving the determination of the influence of the CuII:Mebta ratio, the nature of solvent and the presence of counterions on the identity of the reaction products. As a consequence, complexes [Cu2Cl4(Mebta)4] (1), [CuCl2(Mebta)2] (2), {[Cu2Cl4(Mebta)2]}n (3), [Cu4OCl6(Mebta)4] · 0.25H2O (4 · 0.25H2O) and [Cu2Cl2(Mebta)6](ClO4)2 (5) have been isolated and structurally characterized by single-crystal X-ray studies. Mebta behaves as a monodentate ligand binding through N(3). 1 is a dinuclear complex, the structure of 2 consists of discrete monomeric units, and that of 3 is composed of linear, well-separated polymeric chains of CuII atoms. The molecules of 4 · 0.25H2O have a central μ4-oxide ion surrounded tetrahedrally by four CuII atoms. In the cations of 5 the two CuII centres are asymmetrically bridged by two chloro ligands, with three Mebta molecules completing five coordination at each metal. Complexes were characterized by spectroscopic (IR, far-IR, solution UV/Vis) and thermal decomposition (TG, DTG, and DTA) techniques. Variable-temperature magnetic susceptibility data for 1, 3 and 5 showed intramolecular (1, 5) and intrachain (3) ferromagnetic exchange interactions. Estimates of the Jparameters, experimentally derived, were in close agreement with a new magneto-structural criterion developed by us, holding for bis(μ-chloro) copper(II) dimers. A comparison between the CuCl2/Mebta and CuBr2/Mebta systems is also presented. 相似文献
144.
Dionysiou DD Stamatakos GS Uzunoglu NK Nikita KS Marioli A 《Journal of theoretical biology》2004,230(1):1-20
The aim of this paper is to present the current state of a four-dimensional simulation model of solid tumour growth and response to radiotherapy developed by our group. The most prominent points of the algorithms describing the fundamental biological phenomena involved are outlined. A specific application of the model to a selected clinical case of glioblastoma multiforme is described and comparative studies are performed, using various exploratory values of the model parameters. Qualitative agreement with clinical observations has been achieved. Special emphasis is laid on the variability of radiosensitivity parameters throughout the cell cycle and on the influence of the genetic profile of the tumour on its radiosensitivity. The results of the simulation are three-dimensionally reconstructed. A valuable tool for getting insight into the biology of tumour growth and response to radiotherapy and at the same time an advanced patient specific decision support system is expected to emerge after the completion of the necessary extensive clinical evaluation. 相似文献
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Graça G Goodfellow BJ Barros AS Diaz S Duarte IF Spagou K Veselkov K Want EJ Lindon JC Carreira IM Galhano E Pita C Gil AM 《Molecular bioSystems》2012,8(4):1243-1254
We report on the first untargeted UPLC-MS study of 2nd trimester maternal urine and amniotic fluid (AF), to investigate the possible metabolic effects of fetal malformations (FM), gestational diabetes mellitus (GDM) and preterm delivery (PTD). For fetal malformations, considerable metabolite variations were identified in AF and, to a lesser extent, in urine. Using validated PLS-DA models and statistical correlations between UPLC-MS data and previously acquired NMR data, a metabolic picture of fetal hypoxia, enhanced gluconeogenesis, TCA activity and hindered kidney development affecting FM pregnancies was reinforced. Moreover, changes in carnitine, pyroglutamate and polyols were newly noted, respectively, reflecting lipid oxidation, altered placental amino acid transfer and alterations in polyol pathways. Higher excretion of conjugated products in maternal urine was seen suggesting alterations in conjugation reactions. For the pre-diagnostic GDM group, no significant changes were observed, either considering amniotic fluid or maternal urine, whereas, for the pre-PTD group, some newly observed changes were noted, namely, the decrease of particular amino acids and the increase of an hexose (possibly glucose), suggesting alteration in placental amino acid fluxes and a possible tendency for hyperglycemia. This work shows the potential of UPLC-MS for the study of fetal and maternal biofluids, particularly when used in tandem with comparable NMR data. The important roles played by sampling characteristics (e.g. group dimensions) and the specific experimental conditions chosen for MS methods are discussed. 相似文献
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Konstantina Katsarou Eleni Mitta Eirini Bardani Anastasis Oulas Elena Dadami Kriton Kalantidis 《Molecular Plant Pathology》2019,20(3):432-446
RNA silencing is a universal mechanism involved in development, epigenetic modifications and responses to biotic and abiotic stresses. The major components of this mechanism are Dicer-like (DCL), Argonaute (AGO) and RNA-dependent RNA polymerase (RDR) proteins. Understanding the role of each component is of great scientific and agronomic importance. Plants, including Nicotiana benthamiana, an important plant model, usually possess four DCL proteins, each of which has a specific role, namely being responsible for the production of an exclusive small RNA population. Here, we used RNA interference (RNAi) technology to target DCL proteins and produced single and combinatorial mutants for DCL. We analysed the phenotype for each DCL knockdown plant, together with the small RNA profile, by next-generation sequencing (NGS). We also investigated transgene expression, as well as viral infections, and were able to show that DCL suppression results in distinct developmental defects, changes in small RNA populations, increases in transgene expression and, finally, higher susceptibility in certain RNA viruses. Therefore, these plants are excellent tools for the following: (i) to study the role of DCL enzymes; (ii) to overexpress proteins of interest; and (iii) to understand the complex relationship between the plant silencing mechanism and biotic or abiotic stresses. 相似文献
149.
Johanna Chiche Julie Reverso-Meinietti Annabelle Mouchotte Camila Rubio-Patiño Rana Mhaidly Elodie Villa Jozef P. Bossowski Emma Proics Manuel Grima-Reyes Agnès Paquet Konstantina Fragaki Sandrine Marchetti Josette Briere Damien Ambrosetti Jean-François Michiels Thierry Jo Molina Christiane Copie-Bergman Jacqueline Lehmann-Che Jean-Ehrland Ricci 《Cell metabolism》2019,29(6):1243-1257.e10
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