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Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.
Wen, Xinmei; Tan, Wenzhi; Westergard, Thomas; Krishnamurthy, Karthik; Markandaiah, Shashirekha S; Shi, Yingxiao; Lin, Shaoyu; Shneider, Neil A; Monaghan, John; Pandey, Udai B; Pasinelli, Piera; Ichida, Justin K; Trotti, Davide.
Affiliation
  • Wen X; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Tan W; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Westergard T; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Krishnamurthy K; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Markandaiah SS; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Shi Y; Department of Stem Cell Biology and Regenerative Medicine, Eli and Edythe Broad Center for Regenerative Medicine and Stem Cell Research, University of Southern California, Los Angeles, CA 90089, USA.
  • Lin S; Department of Stem Cell Biology and Regenerative Medicine, Eli and Edythe Broad Center for Regenerative Medicine and Stem Cell Research, University of Southern California, Los Angeles, CA 90089, USA.
  • Shneider NA; Department of Neurology, The Center for Motor Neuron Biology and Disease, Columbia University Medical Center, New York, NY 10032, USA.
  • Monaghan J; Department of Pediatrics, Child Neurology and Neurobiology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, One Children's Hospital Drive, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.
  • Pandey UB; Department of Pediatrics, Child Neurology and Neurobiology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, One Children's Hospital Drive, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.
  • Pasinelli P; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
  • Ichida JK; Department of Stem Cell Biology and Regenerative Medicine, Eli and Edythe Broad Center for Regenerative Medicine and Stem Cell Research, University of Southern California, Los Angeles, CA 90089, USA.
  • Trotti D; Frances and Joseph Weinberg Unit for ALS Research, Department of Neuroscience, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA. Electronic address: davide.trotti@jefferson.edu.
Neuron ; 84(6): 1213-25, 2014 Dec 17.
Article in En | MEDLINE | ID: mdl-25521377
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Full text: 1 Database: MEDLINE Main subject: Antisense Elements (Genetics) / Cell Death / Dipeptides / Frontotemporal Dementia / Amyotrophic Lateral Sclerosis / Neurons Type of study: Prognostic_studies Limits: Animals / Humans Language: En Year: 2014 Type: Article
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Full text: 1 Database: MEDLINE Main subject: Antisense Elements (Genetics) / Cell Death / Dipeptides / Frontotemporal Dementia / Amyotrophic Lateral Sclerosis / Neurons Type of study: Prognostic_studies Limits: Animals / Humans Language: En Year: 2014 Type: Article