FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD

Yusuke Fujioka, Shinsuke Ishigaki, Akio Masuda, Yohei Iguchi, Tsuyoshi Udagawa, Hirohisa Watanabe, Masahisa Katsuno, Kinji Ohno, Gen Sobue

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25 Citations (Scopus)

Abstract

FUS is genetically and pathologically linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). To clarify the RNA metabolism cascade regulated by FUS in ALS/FTLD, we compared the FUS-regulated transcriptome profiles in different lineages of primary cells from the central nervous system. The profiles of FUS-mediated gene expression and alternative splicing in motor neurons were similar to those of cortical neurons, but not to those in cerebellar neurons despite the similarity of innate transcriptome signature. The gene expression profiles in glial cells were similar to those in motor and cortical neurons. We identified certain neurological diseases-associated genes, including Mapt, Stx1a, and Scn8a, among the profiles of gene expression and alternative splicing events regulated by FUS. Thus, FUS-regulated transcriptome profiles in each cell-type may determine cellular fate in association with FUS-mediated ALS/FTLD, and identified RNA targets for FUS could be therapeutic targets for ALS/FTLD.

Original languageEnglish
Article number2388
JournalScientific reports
Volume3
DOIs
Publication statusPublished - 2013 Jan 1
Externally publishedYes

ASJC Scopus subject areas

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    Fujioka, Y., Ishigaki, S., Masuda, A., Iguchi, Y., Udagawa, T., Watanabe, H., Katsuno, M., Ohno, K., & Sobue, G. (2013). FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD. Scientific reports, 3, [2388]. https://doi.org/10.1038/srep02388