Now showing items 1-4 of 4

    • Colocalization of 14-3-3 Proteins with SOD1 in Lewy Body-Like Hyaline Inclusions in Familial Amyotrophic Lateral Sclerosis Cases and the Animal Model 

      Okamoto, Yoko; Shirakashi, Yoshitomo; Ihara, Masafumi; Urushitani, Makoto; Oono, Miki; Kawamoto, Yasuhiro; Yamashita, Hirofumi; Shimohama, Shun; Kato, Shinsuke; Hirano, Asao; Tomimoto, Hidekazu; Ito, Hidefumi; Takahashi, Ryosuke; Feany, Mel B. (Public Library of Science, 2011)
      Background and Purpose: Cu/Zn superoxide dismutase (SOD1) is a major component of Lewy body-like hyaline inclusion (LBHI) found in the postmortem tissue of SOD1-linked familial amyotrophic lateral sclerosis (FALS) patients. ...
    • Electrical impedance myography as a biomarker to assess ALS progression 

      Rutkove, Seward Brian; Caress, James B.; Cartwright, Michael S.; Burns, Ted M.; Warder, Judy; David, William Samuel; Goyal, Namita; Maragakis, Nicholas J.; Clawson, Lora; Benatar, Michael; Usher, Sharon; Sharma, Khema R.; Gautam, Shiva; Narayanaswami, Pushpa; Raynor, Elizabeth M; Watson, Mary Lou; Shefner, Jeremy M. (Informa UK Limited, 2012)
      Electrical impedance myography (EIM), a non-invasive, electrophysiological technique, has preliminarily shown value as an ALS biomarker. Here we perform a multicenter study to further assess EIM’s potential for tracking ...
    • Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss 

      Scekic‐Zahirovic, Jelena; Sendscheid, Oliver; El Oussini, Hajer; Jambeau, Mélanie; Sun, Ying; Mersmann, Sina; Wagner, Marina; Dieterlé, Stéphane; Sinniger, Jérome; Dirrig‐Grosch, Sylvie; Drenner, Kevin; Birling, Marie‐Christine; Qiu, Jinsong; Zhou, Yu; Li, Hairi; Fu, Xiang‐Dong; Rouaux, Caroline; Shelkovnikova, Tatyana; Witting, Anke; Ludolph, Albert C; Kiefer, Friedemann; Storkebaum, Erik; Lagier‐Tourenne, Clotilde; Dupuis, Luc (John Wiley and Sons Inc., 2016)
      Abstract FUS is an RNA‐binding protein involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cytoplasmic FUS‐containing aggregates are often associated with concomitant loss of nuclear FUS. ...
    • A Yeast Model of FUS/TLS-Dependent Cytotoxicity 

      Ju, Shulin; Tardiff, Daniel F.; Han, Haesun; Divya, Kanneganti; Maquat, Lynne E.; Bosco, Daryl A.; Hayward, Lawrence J.; Lindquist, Susan; Weissman, Jonathan S.; Zhong, Quan; Brown, Robert H.; Ringe, Dagmar; Petsko, Gregory A. (Public Library of Science, 2011)
      FUS/TLS is a nucleic acid binding protein that, when mutated, can cause a subset of familial amyotrophic lateral sclerosis (fALS). Although FUS/TLS is normally located predominantly in the nucleus, the pathogenic mutant ...