Journal
STEM CELL RESEARCH
Volume 27, Issue -, Pages 172-179Publisher
ELSEVIER
DOI: 10.1016/j.scr.2018.01.037
Keywords
LncRNAs; Mouse ESCs; Motor neurons; Differentiation; FUS; ALS
Funding
- ERC
- AriSLA
- 'ARCI', MAECI Grant circALS, Telethon [GGP16213]
- Epigen-Epigenomics Flagship Project
- Human Frontiers Science Program Award [RGP0009/2014]
- AFM-Telethon [17835]
- Fondazione Roma NCDs
- NIH/NINDS [R01 NS07377]
- Judith and Jean Pape Adams Foundation
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Long non-coding RNAs (lncRNAs) are currently recognized as crucial players in nervous system development, function and pathology. In Amyotrophic Lateral Sclerosis (ALS), identification of causative mutations in FUS and TDP-43 or hexanucleotide repeat expansion in C9ORF72 point to the essential role of aberrant RNA metabolism in neurodegeneration. In this study, by taking advantage of an in vitro differentiation system generating mouse motor neurons (MNs) from embryonic stem cells, we identified and characterized the long non-coding transcriptome of MNs. Moreover, by using mutant mouse MNs carrying the equivalent of one of the most severe ALS-associated FUS alleles (P517L), we identified lncRNAs affected by this mutation. Comparative analysis with human MNs derived in vitro from induced pluripotent stem cells indicated that candidate lncRNAs are conserved between mouse and human. Our work provides a global view of the long non-coding transcriptome of MN, as a prerequisite toward the comprehension of the still poorly characterized non-coding side of MN physiopathology. (c) 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license.
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