Modelling C9ORF72 hexanucleotide repeat expansion in amyotrophic lateral sclerosis and frontotemporal dementia

Acta Neuropathologica

Volumn 127, Issue 3, 2014, Pages 377-389

  Stepto, Alan ^a   Gallo, Jean Marc ^a   Shaw, Christopher E ^a   Hirth, Frank ^a  

^a KING'S COLLEGE LONDON   (United Kingdom)

Author keywords

Amyotrophic lateral sclerosis (ALS); Chromosome 9 open reading frame 72 (C9ORF72); Dipeptide repeat protein (DPR); Drosophila; Frontotemporal dementia (FTD); Frontotemporal lobar degeneration (FTLD); GGGGCC (G4C2) hexanucleotide repeat expansion; hnRNP; Induced pluripotent stem cells (iPSCs); Mouse; RBP; Repeat associated non ATG (RAN) translation; Repeat associated neurodegenerative disease; RNA foci; RNA toxicity; Sequestration; TDP 43; Zebrafish

Indexed keywords

ADARB2 PROTEIN; COMPLEMENTARY RNA; DENN PROTEIN; HETEROGENEOUS NUCLEAR RIBONUCLEOPROTEIN; PROTEIN; RNA; RNA BINDING PROTEIN; UNCLASSIFIED DRUG;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL CULTURE; APOPTOSIS; C9ORF72 GENE; CYTOTOXICITY TEST; DNA STRUCTURE; DROSOPHILA; FRONTOTEMPORAL DEMENTIA; GENE; GENE EXPRESSION; GENE FUNCTION; GENE INTERACTION; GENE LOSS; GENE OVEREXPRESSION; GENE SILENCING; GENETIC ENGINEERING; GENETIC MODEL; HEXANUCLEOTIDE REPEAT EXPANSION; MOLECULAR MECHANICS; NERVE CELL DIFFERENTIATION; NONHUMAN; NUCLEOTIDE SEQUENCE; PATHOGENESIS; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; PROTEIN AGGREGATION; REVIEW; ZEBRA FISH;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; APOPTOSIS; CELLS, CULTURED; DISEASE MODELS, ANIMAL; DNA REPEAT EXPANSION; FRONTOTEMPORAL DEMENTIA; HUMANS; OPEN READING FRAMES; PROTEINS; RNA;

EID: 84896721441     PISSN: 00016322     EISSN: 14320533     Source Type: Journal    
DOI: 10.1007/s00401-013-1235-1     Document Type: Review

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