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Table 1 ncRNAs and related processes in Huntington’s disease

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

 Nameup/down regulationDescriptionmodelRef
lncRNAHAR1F, HAR1RdownHAR1F and HAR1R are direct targets of REST. Leading to thr repression of several important neuronal genes.human HD brain_striatum[23]
DGCR5downDGCR5 is a REST target lncRNA, which plays an important transcriptional regulatory role in HDhuman HD brain[24]
MEG3downMEG3 is a direct target of REST and found in the chromatin compartment of the cell in association with PRC2 complexhuman HD brain[25, 26]
MEG3, NEAT1upLoss of function of Meg3 and Neat1 modulated mHtt aggregates and downregulation of the Tp53 expressionR6/2 mouse cortex[27]
NEAT1-L, NEAT1-SupNEAT1 provides neuroprotection against mHtt-induced cytotoxicity (NEAT1-L) and oxidative stress-induced injury (NEAT1-S).R6/2 mouse brain[27-29]
TUG1upTUG1 is a direct downstream target of p53 and could modulate mHTT-induced cytotoxicity by p53 activation. TUG1 could be able to binding to the PRC2 epigenetic regulatory complex.human HD brain[30]
TUNAdownTUNA expression was associated with pathological disease severity, decreasing significantly as the disease grade increased.human HD brain_caudate nucleus[31]
LINC00341upunknownhuman HD brain[25]
RPS20P22upRPS20P22 regulates RPS20, which decreases will lead to accumulation of p53.human HD brain[25]
LINC00342downunknownhuman HD brain[25]
NATsAbhd11-osdownAbhd11-os (called Abhd11-AS1 in human) attenuate the mHtt-induced tocixity and contribute to striatal vulnerability in HD.R6/2 mouse model[32]
HTTAS_v1downHttAS-v1 reduces endogenous mHtt tanscript level through RISC-dependent miR-like mechanismhuman HD brain_frontal cortex[7]
BDNF-AS BDNF-AS was associated with the recruitment of polycomb repressive complex 2 (PRC2), which locally induces the trimethylation of histone H3K27 within the locus and in that way plays an important role in the development of HD. [25, 33, 34]
otherssCAGsupsCAG significantly decreased HTT-mediated neuronal viability in an Ago2-dependent mechanism.R6/2 mouse striatum; human HD brain[35]
miRNAsmiR-9*downmiR-9* targets the components of the REST repressor complex, CoREST and modulating the neurotrophic genes expression.HD patient[26]
miR-10b-5pupmiR-10b-5p targets BDNF and correlated to aberrant polycomb repressive complex2 (PRC2) regulation.HD brain_profrontal cortices[36]
miR-22downmiR-22 was found to target HDAC4, Rcor1 (CoREST) and Rgs2 mRNA and achieve neuroprotection and inhibit neurodegeneration.HD brain[26]
miR-27adownmiR-27a increases MDR-1 expression; miR-27a can decrease the mHtt aggregates possibly through MDR-1 modulation.R6/2 mice[37]
miR-34a-5pdownmiR-34a downregulated SIRT1 through binding the 3′ UTR of sirt1 mRNA.R6/2 mice[38]
miR-34bupmiR-34b is a P53-regulated miRNA and the levels may influence mHTT cytoplasmic distribution and toxicityHD patient, plasma[39]
miR-214, miR-125b, miR-146a, miR-150downmiR-214, miR-150, miR-146a and miR-125b target both huamn HTT and mouse Htt.STHdh(Q111)/Hdh(Q111) cells[40]
miR-125b, miR-146a, miR-150downmiR-146a, miR-150 and miR-125b are decreased in striatum of R6/2 mice. miR-125b and miR-150 target p53, which in turn regulates RelA/NFkB and miR-146a expressionsSTHdhQ111/HdhQ111 cells[41]
miR-124downDecreased miR-124 expression could increase CCNA2 and is involved in deregulation of cell cycleSTHdhQ111/HdhQ111 cells[42]
miR-124adownThe neuronal-specific miR-124 is dysregulated in HD tissues, probably as a result of increased repression by REST.R6/2 mouse brain[43]
miR-128adownmiR-128a targets the 3′ UTR of HTT, Huntingtin Interaction Protein 1 (HIP1) and SP1.Transgenic HD monkeys[44]
miR132downThe level of miR-132 associated with Ago2. miR-132 supplementation produced symptomatic improvement of motor function and lifespan.R6/2 mice[45]
miR-212/miR-132downmiR-212/miR-132 clusters are significantly associated with CAG length and implicated in neuronal survival and shows strongest down-regulation in the striatum.HD transgenic mice[35]
miR-196aupmiR-196a might improve mitochondrial function by the upregulation of CBP and PGC-1α to promote oxidation phosphorylation and reduce oxidative stress, which was consistent with the amelioration of cytotoxicity by miR-196a.HD brain, striatum[46]
miR-196aupThe higher expression of RAN binding protein 10 (RANBP10) in the brains of HD transgenic mice and exacerbates neuronal morphology and intracellular transport. miR-196a suppress the expression of RANBP10 through binding to its 3’UTR.HD transgenic mice brain[47]
miR-196aupmiR-196a decreased mHTT expression and pathological aggregates through alteration of several neuronal regulatory pathways including ubiquitin-proteasome systems, gliosis and cAMP response element-binding protein pathway.HD-iPSC derived neurons[48]
miR-19, miR-146a, miR-432downThe increase in the expressions of PCNA, CHEK1 and CCNA2 was found to be the result of decreased expressions of miR-432, miR-146a, and (miR-19a and miR-146a) respectively. Exogenous expressions of these miR-432 and miR-146a in HD cells rescued the abnormalities in cell cycle and apoptosis.STHdh(Q111)/Hdh(Q111) cells[43]