Article
Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations.
Acta neuropathologica - 1 Nov 2012
Neumann Manuela, Valori Chiara F, Ansorge Olaf, Kretzschmar Hans A, Munoz David G, Kusaka Hirofumi, Yokota Osamu, Ishihara Kenji, Ang Lee-Cyn, Bilbao Juan M, Mackenzie Ian R A
Abstract excerpt
Accumulation of the DNA/RNA binding protein fused in sarcoma (FUS) as inclusions in neurons and glia is the pathological hallmark of amyotrophic lateral sclerosis patients with mutations in FUS (ALS-FUS) as well as in several subtypes of frontotemporal lobar degeneration (FTLD-FUS), which are not associated with FUS mutations. Despite some overlap in the phenotype and neuropathology of FTLD-FUS and ALS-FUS,...
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