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Honda D, Ishigaki S, Iguchi Y, et al. The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons. FEBS Open Bio. 2013;4:1-10doi: 10.1016/j.fob.2013.11.001.
Honda, D., Ishigaki, S., Iguchi, Y., Fujioka, Y., Udagawa, T., Masuda, A., Ohno, K., Katsuno, M., & Sobue, G. (2013). The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons. FEBS open bio, 41-10. https://doi.org/10.1016/j.fob.2013.11.001
Honda, Daiyu, et al. "The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons." FEBS open bio vol. 4 (2013): 1-10. doi: https://doi.org/10.1016/j.fob.2013.11.001
Honda D, Ishigaki S, Iguchi Y, Fujioka Y, Udagawa T, Masuda A, Ohno K, Katsuno M, Sobue G. The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons. FEBS Open Bio. 2013 Nov 20;4:1-10. doi: 10.1016/j.fob.2013.11.001. eCollection 2013. PMID: 24319651; PMCID: PMC3851184.
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FEBS Open Bio. 2013 Nov 20;4:1-10. doi: 10.1016/j.fob.2013.11.001. eCollection 2013.

The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.

FEBS open bio

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

Affiliations

  1. Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

PMID: 24319651 PMCID: PMC3851184 DOI: 10.1016/j.fob.2013.11.001

Abstract

TDP-43 and FUS are linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), and loss of function of either protein contributes to these neurodegenerative conditions. To elucidate the TDP-43- and FUS-regulated pathophysiological RNA metabolism cascades, we assessed the differential gene expression and alternative splicing profiles related to regulation by either TDP-43 or FUS in primary cortical neurons. These profiles overlapped by >25% with respect to gene expression and >9% with respect to alternative splicing. The shared downstream RNA targets of TDP-43 and FUS may form a common pathway in the neurodegenerative processes of ALS/FTLD.

Keywords: ALS; ALS, amyotrophic lateral sclerosis; Cugbp1, CUG triplet  repeat, RNA-binding protein 1; DAVID, Database for  Annotation, Visualization and Integrated Discovery; FTLD; FTLD, frontotemporal lobar degeneration; FUS; FUS, fused in sarcoma; GFAP, glial fibrillary acidic protein; GO, Gene Ontology; LTP, long-term potentiation; RIN, RNA integrity numbers; RMA, robust multichip average; RRMs, RNA recognition motifs; SBMA, spinal and bulbar muscular atrophy; TDP-43; TDP-43, transactive response (TAR) DNA-binding protein 43; TGF, transforming growth factor; Transcriptome; hnRNAPs, heterogeneous ribonucleoproteins; shCont, shRNA/control; shCugbp1, shRNA/Cugbp1; shFUS, shRNA/FUS; shTDP, shRNA/TDP-43

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