ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons

DMM Disease Models and Mechanisms

Volumn 8, Issue 7, 2015, Pages 755-766

  Lenzi, Jessica ^a,b   De Santis, Riccardo ^a,b   De Turris, Valeria ^a   Morlando, Mariangela ^b   Laneve, Pietro ^a   Calvo, Andrea ^c   Caliendo, Virginia ^d   Chiò, Adriano ^c   Rosa, Alessandro ^b   Bozzoni, Irene ^a,b,e  

^a ISTITUTO ITALIANO DI TECNOLOGIA   (Italy)

^b SAPIENZA UNIVERSITY OF ROME   (Italy)

^c UNIVERSITY OF TURIN   (Italy)

^d AOU Città della Salute e della Scienza University Hospital   (Italy)

^e SAPIENZA UNIVERSITY OF ROME   (Italy)

Author keywords

ALS; FUS; iPSCs; TALE nucleases

Indexed keywords

FUSED IN SARCOMA PROTEIN; MUTANT PROTEIN; TAR DNA BINDING PROTEIN; TRANSCRIPTION ACTIVATOR LIKE EFFECTOR NUCLEASE; FUS PROTEIN, HUMAN; RNA BINDING PROTEIN FUS;

ADULT; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CASE REPORT; CELL DIFFERENTIATION; CELL FUNCTION; CELL STRESS; CONTROLLED STUDY; FEMALE; FIBROBLAST; GENE MUTATION; HETEROZYGOTE; HOMOZYGOTE; HUMAN; HUMAN CELL; IN VITRO STUDY; MALE; MIDDLE AGED; MOTONEURON; NERVE CELL; NUCLEAR LOCALIZATION SIGNAL; PATHOGENESIS; PHENOTYPE; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; PROTEIN LOCALIZATION; SITE DIRECTED MUTAGENESIS; SPINAL CORD; YOUNG ADULT; ACTIVE TRANSPORT; AMINO ACID SUBSTITUTION; BIOLOGICAL MODEL; CELL GRANULE; CELL LINE; GENETICS; INDUCED PLURIPOTENT STEM CELL; METABOLISM; PATHOLOGY; PHYSIOLOGICAL STRESS;

ACTIVE TRANSPORT, CELL NUCLEUS; AMINO ACID SUBSTITUTION; AMYOTROPHIC LATERAL SCLEROSIS; CELL DIFFERENTIATION; CELL LINE; CYTOPLASMIC GRANULES; HUMANS; INDUCED PLURIPOTENT STEM CELLS; MODELS, NEUROLOGICAL; MOTOR NEURONS; MUTAGENESIS, SITE-DIRECTED; MUTANT PROTEINS; RNA-BINDING PROTEIN FUS; SPINAL CORD; STRESS, PHYSIOLOGICAL;

EID: 84937876670     PISSN: 17548403     EISSN: 17548411     Source Type: Journal    
DOI: 10.1242/dmm.020099     Document Type: Article

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