Table 3.
Therapeutic Potential of Epigenetic Modulators in Huntington’s Disease
Model | Therapeutic Potential | Modification | Citation |
---|---|---|---|
mHTT Drosophila | Selisistat improves survival and decreases cytotoxic inclusions | Histone acetylation | Smith et al., 2014 |
mHTT Drosophila | AK1 and AGK2 are neuroprotective by way of sterol biosynthesis downregulation | Histone acetylation | Luthi-Carter et al., 2010 |
Htn-Q150 C. elegans | Knockdown of HDA3 reduces polyglutamine toxicity | Histone acetylation | Bates et al., 2006 |
Tg-R6/2 mice | HDACi 4b reverses H3 hypoacetylation and improves functional recovery | Histone acetylation | Thomas et al., 2008 |
HTT-Q73 PC12 cells | miR-10b-5p mimic increases cell survival | MiRNA | Hoss et al., 2014 |
HTT-Q84 Neuroblastoma cells | Overexpression of miR-196a increases neurite outgrowth | MiRNA | Fu et al., 2015 |
R6/2 mice | MiR-132 overexpression provided neuroprotection and delayed disease progression | MiRNA | Fukuoka et al., 2018 |
Tg-R6/2 mice | Overexpression of miR-196a enhances neurite outgrowths and improves learning and memory | MiRNA | Her et al., 2017 |
HD neural progenitor cells | Overexpression of miR-196a normalized mitochondrial activity and decreased apoptosis | MiRNA | Kunkanjanawan, et al., 2016 |
STHdh(Q111)/Hdh(Q111) cells | Exogenous expression of miR-146a, miR-432, and miR-19a reversed cell cycle defects and apoptosis | MiRNA | Das et al., 2015 |
STHdhQ7/HdhQ7 cells | Overexpression of HYPK and Hsp70 reverses miR-125b, miR-146a, and miR-150 expressions while reducing mHTT aggregates | MiRNA | Ghose et al., 2011 |
Neuronal stem cells derived from R6/2 mice | MiR-27a overexpression decreased mHTT aggregates | MiRNA | Ban et al., 2017 |
mHTT sheep | HTT targeting by way of artificial miRNA safely reduces mHTT mRNA and protein | MiRNA | Pfister et al., 2018 |
Primary Striatal cell and cortical neurons, mHTT and 3-NP | MiR-22 overexpression is neuroprotective | MiRNA | Jovicic et al., 2013 |
R6/2 neurons | MiR-27a overexpression decreases mHTT aggregates by way of MDR-1 | MiRNA | Ban et al., 2017 |
Neuroblastoma cells, H2O2 | Neat1 transfection increases tolerance to oxidative stress | LncRNA | Sunwoo et al., 2017 |
HEK293 cells and neuroblastoma cells | Overexpression of HTTAS_v1 decreases HTT levels in a gene-specific manner | LncRNA | Chung et al., 2011 |