Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31

Neuron

Volumn 94, Issue 1, 2017, Pages 108-124.e7

  Ishiguro, Taro ^a,b   Sato, Nozomu ^a   Ueyama, Morio ^b,c   Fujikake, Nobuhiro ^b   Sellier, Chantal ^d   Kanegami, Akemi ^e   Tokuda, Eiichi ^f   Zamiri, Bita ^g,h   Gall Duncan, Terence ^g,h   Mirceta, Mila ^g,h   Furukawa, Yoshiaki ^f   Yokota, Takanori ^a   Wada, Keiji ^b   Taylor, J Paul ⁱ   Pearson, Christopher E ^g,h   Charlet Berguerand, Nicolas ^d   Mizusawa, Hidehiro ^a   Nagai, Yoshitaka ^b,c   Ishikawa, Kinya ^a  

^a TOKYO MEDICAL AND DENTAL UNIVERSITY   (Japan)

^b NATIONAL INSTITUTE OF NEUROSCIENCE   (Japan)

^c OSAKA UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^d UNIVERSITÉ DE STRASBOURG   (France)

^e Research Institute of Biomolecule Metrology   (Japan)

^f KEIO UNIVERSITY   (Japan)

^g UNIVERSITY OF TORONTO   (Canada)

^h HOSPITAL FOR SICK CHILDREN   (Canada)

ⁱ ST JUDE CHILDREN S RESEARCH HOSPITAL   (United States)

Author keywords

ALS; Drosophila melanogaster; microsatellite repeat expansion diseases; RAN translation; ribonucleoprotein; RNA chaperone; RNA foci; SCA31; TDP 43

Indexed keywords

BRAIN PROTEIN; CHAPERONE; PENTAPEPTIDE REPEAT PROTEIN; RIBONUCLEOPROTEIN; RNA BINDING PROTEIN FUS; TAR DNA BINDING PROTEIN; UNCLASSIFIED DRUG; DNA BINDING PROTEIN; DROSOPHILA PROTEIN; HETEROGENEOUS NUCLEAR RIBONUCLEOPROTEIN GROUP A B; MICROSATELLITE DNA; REPETITIVE DNA; RNA BINDING PROTEIN; TBPH PROTEIN, DROSOPHILA; TDP-43 PROTEIN, HUMAN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; FEMALE; GENE EXPRESSION; HOMEOSTASIS; HUMAN; HUMAN TISSUE; IN VITRO STUDY; IN VIVO STUDY; MALE; MOLECULAR INTERACTION; MOTOR NEURON DISEASE; NONHUMAN; PROTEIN AGGREGATION; PROTEIN RNA BINDING; PROTEIN SYNTHESIS; RAT; REGULATORY MECHANISM; RNA FOLDING; RNA RECOGNITION MOTIF; RNA TRANSLATION; SPINOCEREBELLAR DEGENERATION; TRANSLATION REGULATION; AGED; ANIMAL; DROSOPHILA MELANOGASTER; GENETICS; MIDDLE AGED; PC12 CELL LINE; TRANSGENIC ANIMAL; VERY ELDERLY;

AGED; AGED, 80 AND OVER; ANIMALS; ANIMALS, GENETICALLY MODIFIED; DNA REPEAT EXPANSION; DNA-BINDING PROTEINS; DROSOPHILA MELANOGASTER; DROSOPHILA PROTEINS; FEMALE; HETEROGENEOUS-NUCLEAR RIBONUCLEOPROTEIN GROUP A-B; HUMANS; MALE; MICROSATELLITE REPEATS; MIDDLE AGED; MOLECULAR CHAPERONES; MOTOR NEURON DISEASE; PC12 CELLS; PROTEIN BIOSYNTHESIS; RATS; RNA FOLDING; RNA-BINDING PROTEIN FUS; RNA-BINDING PROTEINS; SPINOCEREBELLAR ATAXIAS;

EID: 85016041911     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2017.02.046     Document Type: Article

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