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. 2020 Feb 25;27:38. doi: 10.1186/s12929-020-00628-z

Table 2.

Proposed functions of lncRNAs in spinal motor neuron diseases

LncRNA Disease Proposed function Organism/cell models Reference
ATXN2-AS ALS (CUG)n repeat expansions induce neurotoxicity. SK-N-MC neuroblastoma cells and lymphoblastoid cell lines from ALS patients [75]
C9ORF72 antisense RNA ALS Forms RNA foci and repeat-associated non-AUG (RAN) translation generates dipeptides to cause neurotoxicity. Drosophila, Zebrafish/Neuro-2a, mouse primary cortical and motor neurons [91, 96, 134, 138, 151, 155, 161]
NEAT1 ALS Facilitates paraspeckle formation. High levels of NEAT1 trigger neurotoxicity. Mouse/NSC-34 MN-like cells [30, 133]
SMN-AS1 SMA Recruits PRC2 complex to suppress the SMN gene. Mouse/human SMA-iPSC-derived MNs, SMNΔ7 mouse cortical neurons [33, 152]