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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

  Name up/down regulation Description model Ref
lncRNA HAR1F, HAR1R down HAR1F and HAR1R are direct targets of REST. Leading to thr repression of several important neuronal genes. human HD brain_striatum [23]
DGCR5 down DGCR5 is a REST target lncRNA, which plays an important transcriptional regulatory role in HD human HD brain [24]
MEG3 down MEG3 is a direct target of REST and found in the chromatin compartment of the cell in association with PRC2 complex human HD brain [25, 26]
MEG3, NEAT1 up Loss of function of Meg3 and Neat1 modulated mHtt aggregates and downregulation of the Tp53 expression R6/2 mouse cortex [27]
NEAT1-L, NEAT1-S up NEAT1 provides neuroprotection against mHtt-induced cytotoxicity (NEAT1-L) and oxidative stress-induced injury (NEAT1-S). R6/2 mouse brain [27-29]
TUG1 up TUG1 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]
TUNA down TUNA expression was associated with pathological disease severity, decreasing significantly as the disease grade increased. human HD brain_caudate nucleus [31]
LINC00341 up unknown human HD brain [25]
RPS20P22 up RPS20P22 regulates RPS20, which decreases will lead to accumulation of p53. human HD brain [25]
LINC00342 down unknown human HD brain [25]
NATs Abhd11-os down Abhd11-os (called Abhd11-AS1 in human) attenuate the mHtt-induced tocixity and contribute to striatal vulnerability in HD. R6/2 mouse model [32]
HTTAS_v1 down HttAS-v1 reduces endogenous mHtt tanscript level through RISC-dependent miR-like mechanism human 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]
others sCAGs up sCAG significantly decreased HTT-mediated neuronal viability in an Ago2-dependent mechanism. R6/2 mouse striatum; human HD brain [35]
miRNAs miR-9* down miR-9* targets the components of the REST repressor complex, CoREST and modulating the neurotrophic genes expression. HD patient [26]
miR-10b-5p up miR-10b-5p targets BDNF and correlated to aberrant polycomb repressive complex2 (PRC2) regulation. HD brain_profrontal cortices [36]
miR-22 down miR-22 was found to target HDAC4, Rcor1 (CoREST) and Rgs2 mRNA and achieve neuroprotection and inhibit neurodegeneration. HD brain [26]
miR-27a down miR-27a increases MDR-1 expression; miR-27a can decrease the mHtt aggregates possibly through MDR-1 modulation. R6/2 mice [37]
miR-34a-5p down miR-34a downregulated SIRT1 through binding the 3′ UTR of sirt1 mRNA. R6/2 mice [38]
miR-34b up miR-34b is a P53-regulated miRNA and the levels may influence mHTT cytoplasmic distribution and toxicity HD patient, plasma [39]
miR-214, miR-125b, miR-146a, miR-150 down miR-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-150 down miR-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 expressions STHdhQ111/HdhQ111 cells [41]
miR-124 down Decreased miR-124 expression could increase CCNA2 and is involved in deregulation of cell cycle STHdhQ111/HdhQ111 cells [42]
miR-124a down The neuronal-specific miR-124 is dysregulated in HD tissues, probably as a result of increased repression by REST. R6/2 mouse brain [43]
miR-128a down miR-128a targets the 3′ UTR of HTT, Huntingtin Interaction Protein 1 (HIP1) and SP1. Transgenic HD monkeys [44]
miR132 down The 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-132 down miR-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-196a up miR-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-196a up The 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-196a up miR-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-432 down The 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]