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1.
Spontaneous mutations in the EEF1A2 gene cause epilepsy and severe neurological disabilities in children. The crystal structure of eEF1A2 protein purified from rabbit skeletal muscle reveals a post-translationally modified dimer that provides information about the sites of interaction with numerous binding partners, including itself, and maps these mutations onto the dimer and tetramer interfaces. The spatial locations of the side chain carboxylates of Glu301 and Glu374, to which phosphatidylethanolamine is uniquely attached via an amide bond, define the anchoring points of eEF1A2 to cellular membranes and interorganellar membrane contact sites. Additional bioinformatic and molecular modeling results provide novel structural insight into the demonstrated binding of eEF1A2 to SH3 domains, the common MAPK docking groove, filamentous actin, and phosphatidylinositol-4 kinase IIIβ. In this new light, the role of eEF1A2 as an ancient, multifaceted, and articulated G protein at the crossroads of autophagy, oncogenesis and viral replication appears very distant from the “canonical” one of delivering aminoacyl-tRNAs to the ribosome that has dominated the scene and much of the thinking for many decades.  相似文献   
2.
Polyolefins functionalized with diethylmaleate were mixed with poly(vinylchloride) (PVC) in different compositions. Intermolecular interactions involving the carbonyl groups of the side chains of the functionalized polyolefins and methine hydrogens of PVC were investigated by means of infrared spectroscopy. The major flexibility of the ester groups attached to the backbone chains, with respect to polyesters, seems to increase the capability of such groups to interact with groups of more polar polymers, thus allowing prediction of easier miscibility, which however also depends on the starting polyolefin structure and function-alization degree.  相似文献   
3.
An overview about the development of porous bioresorbable composite materials for applications as scaffolds in tissue engineering is presented. A thermally induced phase separation method was developed to fabricate porous foam-like structures of poly(lactide-co-glycolide) (PLGA) containing bioactive glass particle additions (up to 50 wt.%) and exhibiting well-defined, oriented and interconnected porosity. The in vitro bioactivity and the degradability of the composite foams were investigated in contact with phosphate buffer saline (PBS). Weight loss, water absorption and molecular weight measurements were used to monitor the polymer degradation after incubation periods of up to 7 weeks in PBS. It was found that the presence of bioactive glass retards the polymer degradation rate for the time period investigated. The present results show a way of controlling the in vitro degradation behaviour of PLGA porous composite scaffolds by tailoring the concentration of bioactive glass.  相似文献   
4.
Summary The essential oil obtained by steam distillation of cinchona bark (Cinchona spp.,Rubiaceae family) — a plant well known for its pharmacological properties, also employed in the food industry, especially in making liqueurs - was investigated. The oil, of completely unknown chemical composition, was analyzed by GLC and GLC/MS after separation on a silica gel column, and one hundred components were identified. The presence of 2-hexyl-3-methylmaleic anhydride is rather unusual.
Ungewöhnliche ätherische Öle mit aromatischen Eigenschaften. Die flüchtigen Aromastoffe der China-Rinde
Zusammenfassung Es wurde ätherisches Öl analysiert, das durch Wasserdampfdestillation von Chinarinde (Cinchona spp. derRubiaceae-Familie) gewonnen wurde. Diese Pflanze ist aufgrund ihrer pharmakologischen Eigenschaften bekannt und wird auch in der Lebensmittelindustrie verwendet, ganz besonders für die Likör- und Magenbitterherstellung. Die chemische Zusammensetzung dieses Öls war unbekannt. Nach Abtrennung auf einer Kieselgelsäule wurden im Öl mit GLC und GLC/MS etwa 100 Verbindungen identifiziert. Das Vorhandensein von 2-Hexyl-3-methyl-maleinsäureanhydrid ist ziemlich ungewöhnlich.
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Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations of the GLA gene that result in a deficiency of the enzymatic activity of α-galactosidase A and consequent accumulation of glycosphingolipids in body fluids and lysosomes of the cells throughout the body. GB3 accumulation occurs in virtually all cardiac cells (cardiomyocytes, conduction system cells, fibroblasts, and endothelial and smooth muscle vascular cells), ultimately leading to ventricular hypertrophy and fibrosis, heart failure, valve disease, angina, dysrhythmias, cardiac conduction abnormalities, and sudden death. Despite available therapies and supportive treatment, cardiac involvement carries a major prognostic impact, representing the main cause of death in FD. In the last years, knowledge has substantially evolved on the pathophysiological mechanisms leading to cardiac damage, the natural history of cardiac manifestations, the late-onset phenotypes with predominant cardiac involvement, the early markers of cardiac damage, the role of multimodality cardiac imaging on the diagnosis, management and follow-up of Fabry patients, and the cardiac efficacy of available therapies. Herein, we provide a comprehensive and integrated review on the cardiac involvement of FD, at the pathophysiological, anatomopathological, laboratory, imaging, and clinical levels, as well as on the diagnosis and management of cardiac manifestations, their supportive treatment, and the cardiac efficacy of specific therapies, such as enzyme replacement therapy and migalastat.  相似文献   
9.
This study, which deals with the distribution of hydrocarbons in seven types of rabbit tissues, was done for the purpose of providing information that might help shed light on the biological relevance of the hydrocarbons in mammalian metabolism. Liver, kidneys, brain, spleen, skeletal muscle, perinephric adipose, and a sample of blood serum were collected from a single animal for analysis of their hydrocarbon composition. The analytical methodology consisted of solvent extraction, saponification (adipose), elution chromatography on hydrated alumina, and combined gas chromatography-mass spectrometry. Hydrocarbons were detected in all of the tissues examined at concentrations estimated to range from 0.1 to 0.01% of the total lipid extracted. Three quite distinct distribution modes were recognized. The bulk of the identified components consisted of normal, saturated, nonterpenoid hydrocarbons in the C16 to C33 range. Squalene, phytene, phytadiene, and pristane were the only terpenoids detected. Nonterepenoid branched (iso andanteiso) hydrocarbons were identified unequivocally and in significant amounts in the muscle only. The adipose was the only tissue which was relatively rich in monoalkenes, and its overall hydrocarbon composition closely resembled that of the feed. The results of the study are not consistent with metabolic inertness. The observed qualitative and quantitative differences might reflect function and metabolic activities of the individual organs in a way yet to be elucidated. Presented in part at the AOCS Spring Meeting, New Orleans, April, 1976.  相似文献   
10.
Electrophoretic deposition of carbon nanotubes   总被引:1,自引:0,他引:1  
Electrophoretic deposition (EPD) has been gaining increasing interest as an economical and versatile processing technique for the production of novel coatings or films of carbon nanotubes (CNTs) on conductive substrates. The purpose of the paper is to present an up-to-date comprehensive overview of current research progress in the field of EPD of CNTs. The paper specifically reviews the preparation and characterisation of stable CNT suspensions, and the mechanism of the EPD process; it includes discussion of pure CNT coatings and CNT/nanoparticle composite films. A complete discussion of the EPD parameters is presented, including electrode materials, deposition time, electrode separation, deposition voltage and resultant electric field. The paper highlights potential applications of the resulting CNT and CNT/composite structures, in areas such as field emission devices, fuel cells, and supercapacitors.  相似文献   
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