Article
Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.
Molecular neurodegeneration - 10 Oct 2012
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
Abstract excerpt
BACKGROUND: 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 inclusions (NCIs) in ALS patients with FUS mutations. FUS is also a major pathologic marker for a group of less common forms of frontotemporal lobar degeneration (FTLD), which...
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