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Table 4 ncRNAs and related processes in other neurodegenerative diseases

From: Functional roles and networks of non-coding RNAs in the pathogenesis of neurodegenerative diseases

Disease ncRNA class Name up/down regulation Mutation Description Model Ref
ALS NATs IER3-AS, ZBTB11-AS, PAXBP-AS, SNAP25-AS, CKMT2-AS, down, down, up,down, down SALS, TDP43, SOD1, FUS IER3-AS and ZBTB11-AS downregulated in sporadic ALS; PAXBP-AS upregulaed in ALS-FUS; SNAP25-AS downregulated in ALS-FUS; CKMT2-AS downregulated in ALS-SOD1. The mechnisms are still unkown. ALS spinal cord extract, peripheral blood mononuclear cells [140]
LncRNAs ncRNACCND1 up FUS/TLS In response to DNA damage, ncRNACCND1 interactes with FUS and represses CCND1 transcription by enhancing inhibition of CBP and p300 histone acetyltransferase activities.   [137]
tiRNAs (tRNA-derived RNA fragments) up ANG-P112L tiRNAs inhibit translation via its G-guadruplex structure. tiRNAs displace eIF4 from mRNA and stablizes YB-1. tiRNAs promotes the untranslated mRNA for stress granule formation. cell line [136]
Lhx1as, LncMN-1, LncMN2   Fus P517L detected in mouse model without know mechanisms. mouse [138]
MicroRNAs miR-17~92 down SOD1G93A; SOD1L144F miR-17~92 cluster target E3 ubiguitin ligase to regulate PTEN subcellular location via monoubiquitination. miR-17~92/nuclear PTEN regulats motor neuron vulnerability in SOD1ALS. ALS patienst’s iPSC, mouse model [145, 146]
miR-155 up SOD1G93A miR-155 distributes in rodent and patients’ spinal cord. Anti-miR-155 treatement improve survival rate by mainly blocking miR-155 funcion in microglia, astrocyte and neuron. ALS spinal cord, rodent model [147]
miR-155 up SOD1G93A miR-155 regulates survival gene expression in microglia incuding P2ry12, Tmem119, Olfml3, Egr1, Atf3, Jun, Fos, and Mafb and Tgfbr1 . ALS spinal cord, rodent model [22]
miR-206 down   miR-206 contols HDAC4 expression in neuromuscular gene expression and restore the NMJ function. mouse model [149]
miR-218 up SOD1G93A miR-218 can be transported from motor neurons to neighbouring astrocytes and sufficiently downregulates glutamate transporter in astrocytes (excitatory amino acid transport 2 (EAAT2)). Blocking miR-218 with antisense oligonucleotides recover EAAT2 expression and mitigates astrogliosis in mouse brain. mouse model [148]
miR-375-3p down Vps54 tumor suppressor gene NDRG2 and miR-375-3p are dysregulated in sporadic ALS. Upregulated NDRG2 increas ROS formation and further activates p53. Insufficient targeting p53 by miR-365-3p leads to NDRG2 and ROS upregulation. ALS-iPSC [143]
miR-375 down FUSP525L miR-375 targets p53 and ELAVL4, which are upregulated due to loss of FUS function. ALS-iPSC [144]
miR-92a-3p, miR-125b-5p, down   miR-92a-3p and miR-125b-5p target NEFM 3’UTR . ALS spinal cord [142]
miR-124-3p, miR-92a-3p, miR-20b-5p miR-223b-3p, down   miR-124-3p, miR-92a-3p, miR-20b-5p and miR-223b-3p target NEFH 3’UTR. ALS spinal cord [142]
ALS; FTLD LncRNA C9ORF72 (repeat expansion) up hexanucleotide repeat expansion in C9orf72 intron 1 HRE repeats expansion disrupts RAN dependent protein/RNA nucleaocytoplasmic transport by sequestering RNAGAP1 and leads to neurodegeneration. HRE also get translated into toxic dipeptide by interacting with PAC1 depedent translation factor and leads to neurodegeneration. ALS patients’ brain, spinal cord; iPSC, mouse, fly [129, 130]
MALAT1, MEG3   TDP43 In iCLIP data, MALAT1 inteactes with TDP43, and FUS interactes with MEG3. ALS patinets’ tissue extract [132, 133]
NEAT1 up TDP43, FUS NEAT1_2 interacte with TDP43 and FUS by iCLIP data. TDP43 and FUS are recruited to paraspeckle due to interaction with NEAT1_2. ALS patients’ tissue extract [132135]
snRNA U12 snRNA, Hsrw up TDP43 associated with neurodegeneration caused by TDP43 promoted transcrption elongation via interaction with ELL2 in elongation complexes. Fly [131]
SMA snRNA variant of U1 snRNA (vU1) up   variant of U1 snRNA (vU1) is upregulated and affects U1 snRNA expression. The ratio of vU1/U1 increased in SMA-iPSC derived MN compared with control. SMA-iPSC [150]
SCA2 NATs ATXN2-AS    ATXN1-AS transcripts with CAG repeats forms RNA foci and detected in SCA2 cerebellar Purkinje cell. ATXN1-AS and CAG repeats trigger caspase 3/7 dependent apoptosis. SCA2 tissues [151]