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  • 1
    Publikationsdatum: 2015-01-29
    Beschreibung: Article Dominant mutations in the RNA-binding protein FUS/TLS cause amyotrophic lateral sclerosis (ALS), an adult-onset motor neuron degenerative disease. Here, the authors show that ALS-causative FUS/TLS mutations directly bind the SMN and U1-snRNP complexes, producing both loss and gain of function effects on RNA processing. Nature Communications doi: 10.1038/ncomms7171 Authors: Shuying Sun, Shuo-Chien Ling, Jinsong Qiu, Claudio P. Albuquerque, Yu Zhou, Seiya Tokunaga, Hairi Li, Haiyan Qiu, Anh Bui, Gene W. Yeo, Eric J. Huang, Kevin Eggan, Huilin Zhou, Xiang-Dong Fu, Clotilde Lagier-Tourenne, Don W. Cleveland
    Digitale ISSN: 2041-1723
    Thema: Biologie , Chemie und Pharmazie , Allgemeine Naturwissenschaft , Physik
    Publiziert von Springer Nature
    Standort Signatur Erwartet Verfügbarkeit
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  • 2
    Publikationsdatum: 2013-02-20
    Beschreibung: Transactivating response region DNA binding protein (TDP-43) is the major protein component of ubiquitinated inclusions found in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with ubiquitinated inclusions. Two ALS-causing mutants (TDP-43Q331K and TDP-43M337V), but not wild-type human TDP-43, are shown here to provoke age-dependent, mutant-dependent, progressive motor axon...
    Print ISSN: 0027-8424
    Digitale ISSN: 1091-6490
    Thema: Biologie , Medizin , Allgemeine Naturwissenschaft
    Standort Signatur Erwartet Verfügbarkeit
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  • 3
    Publikationsdatum: 2019
    Print ISSN: 1465-7392
    Digitale ISSN: 1476-4679
    Thema: Biologie , Medizin
    Publiziert von Springer Nature
    Standort Signatur Erwartet Verfügbarkeit
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  • 4
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    Unbekannt
    American Association for the Advancement of Science (AAAS)
    Publikationsdatum: 2000-05-08
    Beschreibung: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Williamson, T L -- Corson, L B -- Huang, L -- Burlingame, A -- Liu, J -- Bruijn, L I -- Cleveland, D W -- New York, N.Y. -- Science. 2000 Apr 21;288(5465):399.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/10798964" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): Amyotrophic Lateral Sclerosis/*enzymology/genetics/pathology ; Animals ; Apoptosis ; Cells, Cultured ; Copper/metabolism ; Humans ; Mice ; Motor Neurons/metabolism/*pathology ; Mutation ; Neurofilament Proteins/metabolism ; Nitrates/metabolism ; Superoxide Dismutase/*genetics/*metabolism ; Yeasts/cytology/metabolism ; Zinc/*metabolism/toxicity
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 5
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    Unbekannt
    American Association for the Advancement of Science (AAAS)
    Publikationsdatum: 2001-03-17
    Beschreibung: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Kahana, J A -- Cleveland, D W -- New York, N.Y. -- Science. 2001 Mar 2;291(5509):1718-9.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093-0670, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/11253198" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): Active Transport, Cell Nucleus ; Animals ; Cell Compartmentation ; *Cell Cycle Proteins ; Cell Nucleus/*metabolism ; Centrosome/metabolism ; Chromatin/metabolism ; Cytoplasm/metabolism ; DNA-Binding Proteins/metabolism ; *Guanine Nucleotide Exchange Factors ; Interphase ; Karyopherins ; Meiosis ; Microtubule-Associated Proteins/metabolism ; Microtubules/*metabolism ; Mitosis ; *Neoplasm Proteins ; Nuclear Envelope/metabolism ; Nuclear Proteins/*metabolism ; *Phosphoproteins ; Spindle Apparatus/*metabolism ; Xenopus ; *Xenopus Proteins ; ran GTP-Binding Protein/metabolism
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 6
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    Unbekannt
    Nature Publishing Group (NPG)
    Publikationsdatum: 2008-07-18
    Beschreibung: 〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3625042/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3625042/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Polymenidou, Magdalini -- Cleveland, Don W -- R37 NS027036/NS/NINDS NIH HHS/ -- England -- Nature. 2008 Jul 17;454(7202):284-5. doi: 10.1038/454284a.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/18633404" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): Amyotrophic Lateral Sclerosis/enzymology/genetics/*physiopathology ; Animals ; Humans ; Membrane Proteins/metabolism ; Mice ; Mitochondria/metabolism ; Motor Neurons/pathology ; Mutation ; Superoxide Dismutase/genetics/metabolism
    Print ISSN: 0028-0836
    Digitale ISSN: 1476-4687
    Thema: Biologie , Chemie und Pharmazie , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 7
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    Unbekannt
    American Association for the Advancement of Science (AAAS)
    Publikationsdatum: 1992-02-28
    Beschreibung: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Cleveland, D W -- New York, N.Y. -- Science. 1992 Feb 28;255(5048):1052; author reply 1052-5.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/1546301" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): DNA Fingerprinting/*methods ; Forensic Medicine/*methods ; Humans ; Publishing/standards
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 8
    Publikationsdatum: 2007-05-19
    Beschreibung: Muller et al. (Reports, 27 October 2006, p. 654) proposed a role for microtubule nucleation in mitotic checkpoint signaling. However, their observations of spindle defects and mitotic delay after depletion of gamma-tubulin ring complex (gamma-TuRC) components are fully consistent with activation of the established pathway of checkpoint signaling in response to incomplete or unstable interactions between kinetochores of mitotic chromosomes and spindle microtubules.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Weaver, Beth A A -- Cleveland, Don W -- New York, N.Y. -- Science. 2007 May 18;316(5827):982; author reply 982.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093-0670, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/17510348" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): Animals ; Kinetochores/*physiology ; Microtubule-Associated Proteins/*metabolism ; Microtubules/*metabolism/ultrastructure ; *Mitosis ; Signal Transduction ; Spindle Apparatus/*metabolism ; Tubulin/*metabolism
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 9
    Publikationsdatum: 2013-11-23
    Beschreibung: Gamma-ray burst (GRB) 130427A is one of the most energetic GRBs ever observed. The initial pulse up to 2.5 seconds is possibly the brightest well-isolated pulse observed to date. A fine time resolution spectral analysis shows power-law decays of the peak energy from the onset of the pulse, consistent with models of internal synchrotron shock pulses. However, a strongly correlated power-law behavior is observed between the luminosity and the spectral peak energy that is inconsistent with curvature effects arising in the relativistic outflow. It is difficult for any of the existing models to account for all of the observed spectral and temporal behaviors simultaneously.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Preece, R -- Burgess, J Michael -- von Kienlin, A -- Bhat, P N -- Briggs, M S -- Byrne, D -- Chaplin, V -- Cleveland, W -- Collazzi, A C -- Connaughton, V -- Diekmann, A -- Fitzpatrick, G -- Foley, S -- Gibby, M -- Giles, M -- Goldstein, A -- Greiner, J -- Gruber, D -- Jenke, P -- Kippen, R M -- Kouveliotou, C -- McBreen, S -- Meegan, C -- Paciesas, W S -- Pelassa, V -- Tierney, D -- van der Horst, A J -- Wilson-Hodge, C -- Xiong, S -- Younes, G -- Yu, H-F -- Ackermann, M -- Ajello, M -- Axelsson, M -- Baldini, L -- Barbiellini, G -- Baring, M G -- Bastieri, D -- Bellazzini, R -- Bissaldi, E -- Bonamente, E -- Bregeon, J -- Brigida, M -- Bruel, P -- Buehler, R -- Buson, S -- Caliandro, G A -- Cameron, R A -- Caraveo, P A -- Cecchi, C -- Charles, E -- Chekhtman, A -- Chiang, J -- Chiaro, G -- Ciprini, S -- Claus, R -- Cohen-Tanugi, J -- Cominsky, L R -- Conrad, J -- D'Ammando, F -- de Angelis, A -- de Palma, F -- Dermer, C D -- Desiante, R -- Digel, S W -- Di Venere, L -- Drell, P S -- Drlica-Wagner, A -- Favuzzi, C -- Franckowiak, A -- Fukazawa, Y -- Fusco, P -- Gargano, F -- Gehrels, N -- Germani, S -- Giglietto, N -- Giordano, F -- Giroletti, M -- Godfrey, G -- Granot, J -- Grenier, I A -- Guiriec, S -- Hadasch, D -- Hanabata, Y -- Harding, A K -- Hayashida, M -- Iyyani, S -- Jogler, T -- Johannesson, G -- Kawano, T -- Knodlseder, J -- Kocevski, D -- Kuss, M -- Lande, J -- Larsson, J -- Larsson, S -- Latronico, L -- Longo, F -- Loparco, F -- Lovellette, M N -- Lubrano, P -- Mayer, M -- Mazziotta, M N -- Michelson, P F -- Mizuno, T -- Monzani, M E -- Moretti, E -- Morselli, A -- Murgia, S -- Nemmen, R -- Nuss, E -- Nymark, T -- Ohno, M -- Ohsugi, T -- Okumura, A -- Omodei, N -- Orienti, M -- Paneque, D -- Perkins, J S -- Pesce-Rollins, M -- Piron, F -- Pivato, G -- Porter, T A -- Racusin, J L -- Raino, S -- Rando, R -- Razzano, M -- Razzaque, S -- Reimer, A -- Reimer, O -- Ritz, S -- Roth, M -- Ryde, F -- Sartori, A -- Scargle, J D -- Schulz, A -- Sgro, C -- Siskind, E J -- Spandre, G -- Spinelli, P -- Suson, D J -- Tajima, H -- Takahashi, H -- Thayer, J G -- Thayer, J B -- Tibaldo, L -- Tinivella, M -- Torres, D F -- Tosti, G -- Troja, E -- Usher, T L -- Vandenbroucke, J -- Vasileiou, V -- Vianello, G -- Vitale, V -- Werner, M -- Winer, B L -- Wood, K S -- Zhu, S -- New York, N.Y. -- Science. 2014 Jan 3;343(6166):51-4. doi: 10.1126/science.1242302. Epub 2013 Nov 21.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Space Science, University of Alabama in Huntsville, Huntsville, AL 35899, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/24263132" target="_blank"〉PubMed〈/a〉
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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  • 10
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    Unbekannt
    American Association for the Advancement of Science (AAAS)
    Publikationsdatum: 2005-01-22
    Beschreibung: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Miller, Timothy M -- Cleveland, Don W -- R37 NS027036/NS/NINDS NIH HHS/ -- New York, N.Y. -- Science. 2005 Jan 21;307(5708):361-2.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Ludwig Institute for Cancer Research and the Department of Medicine and Neurosciences, University of California, San Diego, La Jolla, CA 92093, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/15661995" target="_blank"〉PubMed〈/a〉
    Schlagwort(e): Amyotrophic Lateral Sclerosis/*drug therapy ; Animals ; Anti-Bacterial Agents/pharmacology/*therapeutic use ; Brain/metabolism ; Ceftriaxone/pharmacology/*therapeutic use ; Clinical Trials as Topic ; Drug Evaluation, Preclinical ; Excitatory Amino Acid Transporter 2/genetics/metabolism ; Glutamic Acid/metabolism ; Humans ; Mice ; Motor Neurons/physiology ; Neurodegenerative Diseases/*drug therapy ; Spinal Cord/metabolism ; Synapses/physiology ; Synaptic Transmission ; beta-Lactams/pharmacology/*therapeutic use
    Print ISSN: 0036-8075
    Digitale ISSN: 1095-9203
    Thema: Biologie , Chemie und Pharmazie , Informatik , Medizin , Allgemeine Naturwissenschaft , Physik
    Standort Signatur Erwartet Verfügbarkeit
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