Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis

Molecular Neurodegeneration

Volumn 7, Issue 1, 2012, Pages

  Verbeeck, Christophe ^a   Deng, Qiudong ^b   Dejesus Hernandez, Mariely ^a   Taylor, Georgia ^b   Ceballos Diaz, Carolina ^c   Kocerha, Jannet ^b   Golde, Todd ^c   Das, Pritam ^a   Rademakers, Rosa ^a   Dickson, Dennis W ^a   Kukar, Thomas ^b  

^a MAYO GRADUATE SCHOOL   (United States)

^b EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c UNIVERSITY OF FLORIDA COLLEGE OF MEDICINE   (United States)

Author keywords

internexin; Adeno associated virus; Amyotrophic lateral sclerosis; Frontotemporal lobar degeneration; Fused in sarcoma proteinopathies; Neuronal cytoplasmic inclusions; p62 SQSTM1; PABP 1; RNA dysfunction; Stress granules; Transgenic mouse models; Ubiquitin

Indexed keywords

ALPHA INTERNEXIN; FUSED IN SARCOMA PROTEIN; POLYADENYLIC ACID BINDING PROTEIN; PROTEIN P62; TAR DNA BINDING PROTEIN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; APOPTOSIS; ARTICLE; ASTROCYTOSIS; BASOPHIL; CONTROLLED STUDY; CYTOPLASM; DISEASE SEVERITY; DISORDERS OF AMINO ACID AND PROTEIN METABOLISM; EOSINOPHIL; FUS PROTEINOPATHY; GENE MUTATION; IMMUNOHISTOCHEMISTRY; MICROGLIA; MOUSE; NERVE DEGENERATION; NEUROPATHOLOGY; NONHUMAN; PROTEIN EXPRESSION; TRANSGENICS;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BRAIN; DISEASE MODELS, ANIMAL; HUMANS; IMMUNOHISTOCHEMISTRY; INCLUSION BODIES; MICE; MICE, TRANSGENIC; MUTATION; NERVE DEGENERATION; NEURONS; RNA-BINDING PROTEIN FUS;

ADENO-ASSOCIATED VIRUS; MUS; MUS MUSCULUS;

EID: 84867217368     PISSN: None     EISSN: 17501326     Source Type: Journal    
DOI: 10.1186/1750-1326-7-53     Document Type: Article

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