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  • 1
    Publication Date: 2015-11-21
    Description: Mucopolysaccharidosis-I (MPS-I) is a lysosomal storage disease (LSD) caused by inactivating mutations of IDUA , encoding the glycosaminoglycan-degrading enzyme α-l-iduronidase. Although MPS-I is associated with skeletal abnormalities, the impact of IDUA deficiency on bone remodeling is poorly defined. Here we report that Idua -deficient mice progressively develop a high bone mass phenotype with pathological lysosomal storage in cells of the osteoblast lineage. Histomorphometric quantification identified shortening of bone-forming units and reduced osteoclast numbers per bone surface. This phenotype was not transferable into wild-type mice by bone marrow transplantation (BMT). In contrast, the high bone mass phenotype of Idua -deficient mice was prevented by BMT from wild-type donors. At the cellular level, BMT did not only normalize defects of Idua -deficient osteoblasts and osteocytes but additionally caused increased osteoclastogenesis. Based on clinical observations in an individual with MPS-I, previously subjected to BMT and enzyme replacement therapy (ERT), we treated Idua -deficient mice accordingly and found that combining both treatments normalized all histomorphometric parameters of bone remodeling. Our results demonstrate that BMT and ERT profoundly affect skeletal remodeling of Idua -deficient mice, thereby suggesting that individuals with MPS-I should be monitored for their bone remodeling status, before and after treatment, to avoid long-term skeletal complications.
    Print ISSN: 0964-6906
    Electronic ISSN: 1460-2083
    Topics: Biology , Medicine
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  • 2
    Publication Date: 2016-09-18
    Description: The increasing application of soil metaproteomics is providing unprecedented, in-depth characterization of the composition and functionality of in situ microbial communities. Despite recent advances in high-resolution mass spectrometry, soil metaproteomics still suffers from a lack of effective and reproducible protein extraction protocols and standardized data analyses. This review discusses the opportunities and limitations of selected techniques in soil-, and leaf litter metaproteomics, and presents a step-by-step guideline on their application, covering sampling, sample preparation, extraction and data evaluation strategies. In addition, we present recent applications of soil metaproteomics and discuss how such approaches, linking phylogenetics and functionality, can help gain deeper insights into terrestrial microbial ecology. Finally, we strongly recommend that to maximize the insights environmental metaproteomics may provide, such methods should be employed within a holistic experimental approach considering relevant aboveground and belowground ecosystem parameters.
    Print ISSN: 0168-6496
    Electronic ISSN: 1574-6941
    Topics: Biology
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  • 3
    Publication Date: 2013-10-26
    Description: Environmental Science & Technology DOI: 10.1021/es4021074
    Print ISSN: 0013-936X
    Electronic ISSN: 1520-5851
    Topics: Chemistry and Pharmacology , Energy, Environment Protection, Nuclear Power Engineering
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  • 4
    Publication Date: 2012-02-16
    Description: SUMMARY Antarctica represents a key component in the investigation of the geological history and reconstruction of the supercontinents Rodinia and Gondwana. Remnants of the formation and disintegration of these ancient land masses can be found, although great uncertainties remain in the location of tectonic boundaries beneath the ice sheet of Antarctica due to general lack of outcrops and the limited amount of geological data. Airborne and space measurements are the only possibility to obtain comprehensive spatial data coverage for geological studies in the remote polar areas. More than 100 000 line kilometres of airborne geophysical data, including radio echo sounding, gravity and magnetic data, were acquired over an area of 1.2 million square kilometres in the centre of Dronning Maud Land (DML) over four austral summer campaigns between 2001 and 2005. The data are presented here as compilations of homogeneous topographic and gravity data for the DML region, ranging from 14°W to 20°E and from 70°S to 78.5°S. With respect to older airborne geophysical investigations in DML, up to 85 per cent of the gravity data cover unexplored regions. Analyses of the maps of topography and gravity anomalies, by filtering and isostatic analysis, reveal information about geological and tectonic structures in DML. Different gravity maps provide hints for the general tectonic fabric of the area. For the first time the southern boundary of the continent–ocean boundary formed during the Jurassic dispersal of Gondwana is identified. Especially the mountain range in DML is most likely not in isostatic balance. The area is still uplifting as a consequence of glacial rebound. Interpretations of the Jutul–Penck Graben as failed Jurassic rift system can be confirmed by the gravity inversion. Thinned continental crust compared to the surrounding geological units strongly support this hypothesis. Besides on other interpreted anomalies, the data show a gravity structure, which starts approximately at 73.25°S/006°E and strikes in southwestern direction. We speculate that this pattern represents the suture zone [eastern termination of East African–Antarctic Orogen (EAAO)] between southern Africa and the cratonic part of Antarctica.
    Print ISSN: 0956-540X
    Electronic ISSN: 1365-246X
    Topics: Geosciences
    Published by Oxford University Press on behalf of The Deutsche Geophysikalische Gesellschaft (DGG) and the Royal Astronomical Society (RAS).
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  • 5
    Publication Date: 2014-10-25
    Description: One of the most important classifications in chemistry and within the periodic table is the concept of formal oxidation states. The preparation and characterization of compounds containing elements with unusual oxidation states is of great interest to chemists. The highest experimentally known formal oxidation state of any chemical element is at present VIII, although higher oxidation states have been postulated. Compounds with oxidation state VIII include several xenon compounds (for example XeO4 and XeO3F2) and the well-characterized species RuO4 and OsO4 (refs 2-4). Iridium, which has nine valence electrons, is predicted to have the greatest chance of being oxidized beyond the VIII oxidation state. In recent matrix-isolation experiments, the IrO4 molecule was characterized as an isolated molecule in rare-gas matrices. The valence electron configuration of iridium in IrO4 is 5d(1), with a formal oxidation state of VIII. Removal of the remaining d electron from IrO4 would lead to the iridium tetroxide cation ([IrO4](+)), which was recently predicted to be stable and in which iridium is in a formal oxidation state of IX. There has been some speculation about the formation of [IrO4](+) species, but these experimental observations have not been structurally confirmed. Here we report the formation of [IrO4](+) and its identification by infrared photodissociation spectroscopy. Quantum-chemical calculations were carried out at the highest level of theory that is available today, and predict that the iridium tetroxide cation, with a Td-symmetrical structure and a d(0) electron configuration, is the most stable of all possible [IrO4](+) isomers.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Wang, Guanjun -- Zhou, Mingfei -- Goettel, James T -- Schrobilgen, Gary J -- Su, Jing -- Li, Jun -- Schloder, Tobias -- Riedel, Sebastian -- England -- Nature. 2014 Oct 23;514(7523):475-7. doi: 10.1038/nature13795.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Collaborative Innovation Center of Chemistry for Energy Materials, Department of Chemistry, Shanghai Key Laboratory of Molecular Catalysts and Innovative Materials, Fudan University, Shanghai 200433, China. ; Department of Chemistry, McMaster University, 1280 Main Street West, Hamilton, Ontario L8S 4M1, Canada. ; Department of Chemistry and Key Laboratory of Organic Optoelectronics and Molecular Engineering of Ministry of Education, Tsinghua University, Beijing 100084, China. ; Institut fur Anorganische und Analytische Chemie, Albert-Ludwigs Universitat Freiburg, Albertstrasse 21, D-79104 Freiburg im Breisgau, Germany. ; 1] Institut fur Anorganische und Analytische Chemie, Albert-Ludwigs Universitat Freiburg, Albertstrasse 21, D-79104 Freiburg im Breisgau, Germany [2] Institut fur Chemie und Biochemie - Anorganische Chemie, Freie Universitat Berlin, Fabeckstrasse 34-36, D-14195 Berlin, Germany.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/25341786" target="_blank"〉PubMed〈/a〉
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 6
    Publication Date: 2015-04-04
    Description: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Heilmann, Stefanie -- Drichel, Dmitriy -- Clarimon, Jordi -- Fernandez, Victoria -- Lacour, Andre -- Wagner, Holger -- Thelen, Mathias -- Hernandez, Isabel -- Fortea, Juan -- Alegret, Montserrat -- Blesa, Rafael -- Mauleon, Ana -- Roca, Maitee Rosende -- Kornhuber, Johannes -- Peters, Oliver -- Heun, Reinhard -- Frolich, Lutz -- Hull, Michael -- Heneka, Michael T -- Ruther, Eckart -- Riedel-Heller, Steffi -- Scherer, Martin -- Wiltfang, Jens -- Jessen, Frank -- Becker, Tim -- Tarraga, Lluis -- Boada, Merce -- Maier, Wolfgang -- Lleo, Alberto -- Ruiz, Agustin -- Nothen, Markus M -- Ramirez, Alfredo -- England -- Nature. 2015 Apr 2;520(7545):E3-5. doi: 10.1038/nature14039.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉1] Institute of Human Genetics, University of Bonn, 53127 Bonn, Germany [2] Department of Genomics, Life &Brain Center, University of Bonn, 53127 Bonn, Germany. ; German Center for Neurodegenerative Diseases (DZNE), 53175 Bonn, Germany. ; 1] Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital Santa Creu i Sant Pau, Autonomous University Barcelona, 08025 Barcelona, Spain [2] Center for Networking Biomedical Research in Neurodegenerative Diseases (CIBERNED), 28029 Madrid, Spain. ; Memory Clinic of Fundacio ACE, Catalan Institute of Applied Neurosciences, 08028 Barcelona, Spain. ; Department of Psychiatry and Psychotherapy, University of Bonn, 53127 Bonn, Germany. ; Department of Psychiatry and Psychotherapy, University Clinic Erlangen, Friedrich-Alexander University Erlangen-Nurnberg, 91054 Erlangen, Germany. ; Department of Psychiatry, Charite University Medicine, 14050 Berlin, Germany. ; Department of Geriatric Psychiatry, Central Institute of Mental Health, Medical Faculty Mannheim, University of Heidelberg, 68159 Mannheim, Germany. ; Centre for Geriatric Medicine and Section of Gerontopsychiatry and Neuropsychology, Medical School, University of Freiburg, 79106 Freiburg, Germany. ; 1] German Center for Neurodegenerative Diseases (DZNE), 53175 Bonn, Germany [2] Clinical Neuroscience Unit, Department of Neurology, University of Bonn, 53127 Bonn, Germany. ; Department of Psychiatry and Psychotherapy, University of Gottingen, 37075 Gottingen, Germany. ; Institute of Social Medicine, Occupational Health and Public Health, University of Leipzig, 04103 Leipzig, Germany. ; Department of Primary Medical Care, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany. ; 1] German Center for Neurodegenerative Diseases (DZNE), 53175 Bonn, Germany [2] Department of Psychiatry and Psychotherapy, University of Bonn, 53127 Bonn, Germany [3] Department of Psychiatry and Psychotherapy, University of Cologne, 50937 Cologne, Germany. ; 1] German Center for Neurodegenerative Diseases (DZNE), 53175 Bonn, Germany [2] Institute for Medical Biometry, Informatics and Epidemiology, University of Bonn, 53127 Bonn, Germany. ; 1] German Center for Neurodegenerative Diseases (DZNE), 53175 Bonn, Germany [2] Department of Psychiatry and Psychotherapy, University of Bonn, 53127 Bonn, Germany. ; 1] Institute of Human Genetics, University of Bonn, 53127 Bonn, Germany [2] Department of Psychiatry and Psychotherapy, University of Bonn, 53127 Bonn, Germany.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/25832411" target="_blank"〉PubMed〈/a〉
    Keywords: Alzheimer Disease/*genetics ; Female ; Genetic Predisposition to Disease/*genetics ; Genetic Variation/*genetics ; Humans ; Male ; Phospholipase D/*genetics
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 7
    Publication Date: 2019
    Description: 〈p〉The habenula, an epithalamic nucleus involved in reward and aversive processing, may contribute to negative reinforcement mechanisms maintaining nicotine use. We used a performance feedback task that differentially activates the striatum and habenula and administered nicotine and varenicline (versus placebos) to overnight-abstinent smokers and nonsmokers to delineate feedback-related functional brain alterations both as a function of smoking trait (smokers versus nonsmokers) and drug administration state (drug versus placebo). Smokers showed less striatal responsivity to positive feedback, an alteration not mitigated by drug administration, but rather correlated with trait-level addiction severity. Conversely, nicotine administration reduced habenula activity following both positive and negative feedback among abstinent smokers, but not nonsmokers, and increased habenula activity among smokers correlated with elevated state-level tobacco cravings. These outcomes highlight a dissociation between neurobiological processes linked with the dependence severity trait and the nicotine withdrawal state. Interventions simultaneously targeting both aspects may improve currently poor cessation outcomes.〈/p〉
    Electronic ISSN: 2375-2548
    Topics: Natural Sciences in General
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  • 8
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Biomembranes 557 (1979), S. 179-187 
    ISSN: 0005-2736
    Keywords: (Brush border membrane) ; Detergent solubilization ; Octyl glucoside
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Biomembranes 640 (1981), S. 43-54 
    ISSN: 0005-2736
    Keywords: (Brush border membrane) ; Cotransport ; Glucose ; Membrane protein ; Na^+ ; Phlorizin polymer
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Weinheim : Wiley-Blackwell
    Acta Polymerica 34 (1983), S. 328-331 
    ISSN: 0323-7648
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Physics
    Description / Table of Contents: 2,2′-azo-bis-isobutyronitrile was completely thermally decomposed in allyl benzene. The reaction mixture obtained was separated by preparative gas and thin-layer chromatography. Using steam pressure measurements and the logarithmic dependence of gas chromatographic retention value of the molecular weight the molecular weight of the separated components was investigated. The structure was analysed by IR- and NMR-spectroscopy and 14C-labelled tracer substances. A mechanism of the reactions is discussed.
    Notes: 2,2′-Azo-bis-isobutyronitril wurde in Allylbenzen thermisch vollständig zersetzt. Das entstehende Reaktionsgemisch konnte mit Hilfe der präparativen Gas- und Dünnschichtchromatographie getrennt werden. Die Bestimmung der Molmassen der abgetrennten Komponenten erfolgte über Dampfdruckmessungen sowie über die logarithmische Abhängigkeit der gaschromatographischen Retentionswerte von den Molmassen. Die Konstitutions- und Konfigurationsaufklärung gelang mittels IR- und NMR-Spektroskopie sowie durch Anwendung von 14C-markierten Tracer-Substanzen. Ein möglicher Reaktionsablauf wird diskutiert.
    Additional Material: 1 Ill.
    Type of Medium: Electronic Resource
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