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Table 2 Proposed functions of lncRNAs in spinal motor neuron diseases

From: Functional Roles of Long Non-coding RNAs in Motor Neuron Development and Disease

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]