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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.
  • 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 en En | MEDLINE | ID: mdl-25521377

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Elementos sin Sentido (Genética) / Muerte Celular / Dipéptidos / Demencia Frontotemporal / Esclerosis Amiotrófica Lateral / Neuronas Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Año: 2014 Tipo del documento: Article

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Elementos sin Sentido (Genética) / Muerte Celular / Dipéptidos / Demencia Frontotemporal / Esclerosis Amiotrófica Lateral / Neuronas Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Año: 2014 Tipo del documento: Article