Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate invitro and invivo neuronal death

Neuron

Volumn 84, Issue 6, 2014, Pages 1213-1225

  Wen, Xinmei ^a   Tan, Wenzhi ^a   Westergard, Thomas ^a   Krishnamurthy, Karthik ^a   Markandaiah, Shashirekha S ^a   Shi, Yingxiao ^b   Lin, Shaoyu ^b   Shneider, Neil A ^c   Monaghan, John ^d   Pandey, Udai B ^d   Pasinelli, Piera ^a   Ichida, Justin K ^b   Trotti, Davide ^a  

^a THOMAS JEFFERSON UNIVERSITY   (United States)

^b UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^c NewYork Presbyterian Hospital   (United States)

^d CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ARGININE; BRAIN PROTEIN; DIPEPTIDE; PROLINE; PROTEIN C9ORF72; UNCLASSIFIED DRUG; C9ORF72 PROTEIN, HUMAN; NUCLEIC ACID PROBE; PROTEIN; REPETITIVE DNA;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; C9ORF72 GENE; CELL SURVIVAL; CONTROLLED STUDY; EMBRYO; FRONTOTEMPORAL DEMENTIA; GENE EXPRESSION REGULATION; HUMAN; HUMAN TISSUE; IN VITRO STUDY; IN VIVO STUDY; NERVE CELL CULTURE; NERVE CELL NECROSIS; NEUROTOXICITY; NONHUMAN; NUCLEOLUS; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; RAT; RNA PROCESSING; RNA SEQUENCE; TRANSCRIPTION REGULATION; TRANSLATION REGULATION; ANIMAL; CELL DEATH; DROSOPHILA MELANOGASTER; DRUG EFFECTS; GENETICS; METABOLISM; MOTONEURON; NERVE CELL; NUCLEIC ACID PROBE; PATHOLOGY; PRIMARY CELL CULTURE; PROTEINOSIS; SPINAL CORD; TOXICITY; TRANSGENIC ANIMAL;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; ANIMALS, GENETICALLY MODIFIED; ANTISENSE ELEMENTS (GENETICS); ARGININE; CELL DEATH; DIPEPTIDES; DNA REPEAT EXPANSION; DROSOPHILA MELANOGASTER; FRONTOTEMPORAL DEMENTIA; HUMANS; MOTOR NEURONS; NEURONS; PRIMARY CELL CULTURE; PROLINE; PROTEIN AGGREGATION, PATHOLOGICAL; PROTEINS; SPINAL CORD;

EID: 84926357619     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2014.12.010     Document Type: Article

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