Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

Neuron

Volumn 88, Issue 5, 2015, Pages 902-909

  Peters, Owen M ^a   Cabrera, Gabriela Toro ^a   Tran, Helene ^a   Gendron, Tania F ^b   McKeon, Jeanne E ^a   Metterville, Jake ^a   Weiss, Alexandra ^a   Wightman, Nicholas ^a   Salameh, Johnny ^a   Kim, Juhyun ^f   Sun, Huaming ^a   Boylan, Kevin B ^c   Dickson, Dennis ^b   Kennedy, Zachary ^a   Lin, Ziqiang ^a   Zhang, Yong Jie ^b   Daughrity, Lillian ^b   Jung, Chris ^e   Gao, Fen Biao ^a   Sapp, Peter C ^a,d   Horvitz, H Robert ^d   Bosco, Daryl A ^a   Brown, Solange P ^f   de Jong, Pieter ^e   Petrucelli, Leonard ^b   Mueller, Christian ^a   Brown, Robert H ^a   more..

^a UNIVERSITY OF MASSACHUSETTS MEDICAL SCHOOL   (United States)

^b MAYO GRADUATE SCHOOL   (United States)

^c MAYO CLINIC   (United States)

^d MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

^e CHILDREN S HOSPITAL OAKLAND RESEARCH INSTITUTE   (United States)

^f JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Amyotrophic lateral sclerosis (ALS); C9ORF72; Frontotemporal dementia (FTD); MicroRNA; Neurodegeneration; RAN translation; Repeat expansions; RNA foci; Transgenic mice

Indexed keywords

CARRIER PROTEINS AND BINDING PROTEINS; DIPEPTIDE; DIPEPTIDE REPEAT PROTEIN; MICRORNA; POLYGLYCYLPROLINE; RNA; UNCLASSIFIED DRUG; C9ORF72 PROTEIN, HUMAN; NERVE PROTEIN; PROTEIN; REPETITIVE DNA;

ADULT; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BACTERIAL ARTIFICIAL CHROMOSOME; C9ORF72 GENE; CONTROLLED STUDY; DNA STRUCTURE; EXON; FRONTOTEMPORAL DEMENTIA; GENE; GENE SILENCING; HEXANUCLEOTIDE REPEAT; HISTOPATHOLOGY; IN VITRO STUDY; MALE; MOUSE; NERVE CELL CULTURE; NEWBORN; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; TRANSGENE; AGE; ANIMAL; BRAIN; BRAIN CORTEX; CELL CULTURE; CYTOLOGY; DISEASE MODEL; DRUG EFFECTS; GENE EXPRESSION REGULATION; GENETICS; GENOTYPE; HUMAN; METABOLISM; MORTALITY; NERVE CELL; PATHOLOGY; PHYSIOLOGY; TRANSGENIC MOUSE;

AGE FACTORS; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BRAIN; CELLS, CULTURED; CEREBRAL CORTEX; CHROMOSOMES, ARTIFICIAL, BACTERIAL; DIPEPTIDES; DISEASE MODELS, ANIMAL; DNA REPEAT EXPANSION; FRONTOTEMPORAL DEMENTIA; GENE EXPRESSION REGULATION; GENOTYPE; HUMANS; IN VITRO TECHNIQUES; MICE, TRANSGENIC; MICRORNAS; NERVE TISSUE PROTEINS; NEURONS; PROTEINS;

EID: 84949445156     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2015.11.018     Document Type: Article

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