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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

LncRNADiseaseProposed functionOrganism/cell modelsReference
ATXN2-ASALS(CUG)n repeat expansions induce neurotoxicity.SK-N-MC neuroblastoma cells and lymphoblastoid cell lines from ALS patients[75]
C9ORF72 antisense RNAALSForms 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]
NEAT1ALSFacilitates paraspeckle formation. High levels of NEAT1 trigger neurotoxicity.Mouse/NSC-34 MN-like cells[30, 133]
SMN-AS1SMARecruits PRC2 complex to suppress the SMN gene.Mouse/human SMA-iPSC-derived MNs, SMNΔ7 mouse cortical neurons[33, 152]