Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

Acta Neuropathologica

Volumn 128, Issue 4, 2014, Pages 505-524

  Zhang, Yong Jie ^a   Jansen West, Karen ^a   Xu, Ya Fei ^a   Gendron, Tania F ^a   Bieniek, Kevin F ^a,b   Lin, Wen Lang ^a   Sasaguri, Hiroki ^a   Caulfield, Thomas ^a   Hubbard, Jaime ^a   Daughrity, Lillian ^a   Chew, Jeannie ^a,b   Belzil, Veronique V ^a   Prudencio, Mercedes ^a   Stankowski, Jeannette N ^a   Castanedes Casey, Monica ^a   Whitelaw, Ena ^a   Ash, Peter E A ^c   DeTure, Michael ^a   Rademakers, Rosa ^a   Boylan, Kevin B ^a   Dickson, Dennis W ^a   Petrucelli, Leonard ^a   more..

^a MAYO CLINIC   (United States)

^b MAYO CLINIC   (United States)

^c BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Amyotrophic lateral sclerosis; C9ORF72; ER stress; Expanded repeat; Frontotemporal dementia; Poly(GA) proteins; Proteasome activity; Repeat associated non ATG translation

Indexed keywords

CASPASE 3; MOLECULAR LAYER; POLY(GA) RAN TRANSLATED PROTEIN; PROTEASOME; RAN PROTEIN; RECOMBINANT PROTEIN; SALUBRINAL; UNCLASSIFIED DRUG; ANTIBODY; C9ORF72 PROTEIN, HUMAN; CHOLAGOGUE; NERVE PROTEIN; PROTEIN; REPETITIVE DNA;

AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CELL CULTURE; CELL INCLUSION; CELL NUCLEUS INCLUSION BODY; CEREBELLUM; CONTROLLED STUDY; DENTATE NUCLEUS; ENDOPLASMIC RETICULUM STRESS; ENZYME ACTIVATION; FASCIA DENTATA; FEMALE; FRONTAL CORTEX; FRONTAL LOBE; FRONTOTEMPORAL DEMENTIA; GRANULE CELL; HIPPOCAMPUS; HUMAN; HUMAN CELL; HUMAN TISSUE; IMMUNOFLUORESCENCE; IMMUNOHISTOCHEMISTRY; MALE; MOLECULAR WEIGHT; NERVE CELL; NERVE CELL CULTURE; NERVE CELL NECROSIS; NERVE FIBER GROWTH; NEUROTOXICITY; PATHOGENESIS; PROTEIN AGGREGATION; PROTEIN EXPRESSION; STOP CODON; SUBTHALAMIC NUCLEUS; TEMPORAL CORTEX; THALAMUS; ADULT; AGED; ANIMAL; ANIMAL EMBRYO; BRAIN; CHEMISTRY; DRUG EFFECTS; GENE EXPRESSION REGULATION; GENETICS; HEK293 CELL LINE; IMMUNOLOGY; METABOLISM; MIDDLE AGED; MOUSE; NUCLEOLUS; PATHOLOGY; PHYSIOLOGY; PROTEIN SECONDARY STRUCTURE; RADIATION RESPONSE; ULTRASTRUCTURE; VERY ELDERLY;

ADULT; AGED; AGED, 80 AND OVER; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; ANTIBODIES; BRAIN; CELL NUCLEOLUS; CELLS, CULTURED; CHOLAGOGUES AND CHOLERETICS; DNA REPEAT EXPANSION; EMBRYO, MAMMALIAN; ENDOPLASMIC RETICULUM STRESS; FEMALE; FRONTOTEMPORAL DEMENTIA; GENE EXPRESSION REGULATION; HEK293 CELLS; HUMANS; MALE; MICE; MIDDLE AGED; NERVE TISSUE PROTEINS; PROTEIN STRUCTURE, SECONDARY; PROTEINS;

EID: 84919912448     PISSN: 00016322     EISSN: 14320533     Source Type: Journal    
DOI: 10.1007/s00401-014-1336-5     Document Type: Article

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