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

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

<p>Abstract</p> <p>Background</p> <p>Mutations in the gene encoding the RNA-binding protein fused in sarcoma (FUS) can cause familial and sporadic amyotrophic lateral sclerosis (ALS) and rarely frontotemproal dementia (FTD). FUS accumulates in neuronal cytoplasmic inclu...

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Bibliographic Details
Main Authors: Verbeeck Christophe, Deng Qiudong, DeJesus-Hernandez Mariely, Taylor Georgia, Ceballos-Diaz Carolina, Kocerha Jannet, Golde Todd, Das Pritam, Rademakers Rosa, Dickson Dennis W, Kukar Thomas
Format: Article
Language:English
Published: BMC 2012-10-01
Series:Molecular Neurodegeneration
Subjects:
Amyotrophic lateral sclerosis
Frontotemporal lobar degeneration
Fused in sarcoma proteinopathies
Transgenic mouse models
Adeno-associated virus
Neuronal cytoplasmic inclusions
Ubiquitin
p62/SQSTM1
α-internexin
PABP-1
Stress granules
RNA dysfunction
Online Access:http://www.molecularneurodegeneration.com/content/7/1/53

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http://www.molecularneurodegeneration.com/content/7/1/53

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