Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

Martinez, Bridget; Peplow, Philip V.

doi:10.4103/1673-5374.310673

Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

Journal Article · Mon Nov 01 00:00:00 EDT 2021 · Neural Regeneration Research

DOI:https://doi.org/10.4103/1673-5374.310673· OSTI ID:2470431

Martinez, Bridget ^[1]; Peplow, Philip V. ^[2]

Los Alamos National Laboratory (LANL), Los Alamos, NM (United States); St. Georges University School of Medicine (Grenada)
University of Otago, Dunedin (New Zealand)

A review of recent animal models of Huntington’s disease showed many microRNAs had altered expression levels in the striatum and cerebral cortex, and which were mostly downregulated. Among the altered microRNAs were miR-9/9*, miR-29b, miR-124a, miR-132, miR-128, miR-139, miR-122, miR-138, miR-23b, miR-135b, miR-181 (all downregulated) and miR-448 (upregulated), and similar changes had been previously found in Huntington’s disease patients. In the animal cell studies, the altered microRNAs included miR-9, miR-9*, miR-135b, miR-222 (all downregulated) and miR-214 (upregulated). In the animal models, overexpression of miR-155 and miR-196a caused a decrease in mutant huntingtin mRNA and protein level, lowered the mutant huntingtin aggregates in striatum and cortex, and improved performance in behavioral tests. Improved performance in behavioral tests also occurred with overexpression of miR-132 and miR-124. In the animal cell models, overexpression of miR-22 increased the viability of rat primary cortical and striatal neurons infected with mutant huntingtin and decreased huntingtin -enriched foci of ≥ 2 µm. Also, overexpression of miR-22 enhanced the survival of rat primary striatal neurons treated with 3-nitropropionic acid. Exogenous expression of miR-214, miR-146a, miR-150, and miR-125b decreased endogenous expression of huntingtin mRNA and protein in Hdh^Q111 /Hdh^Q111 cells. Further studies with animal models of Huntington’s disease are warranted to validate these findings and identify specific microRNAs whose overexpression inhibits the production of mutant huntingtin protein and other harmful processes and may provide a more effective means of treating Huntington’s disease in patients and slowing its progression.

View Accepted Manuscript (DOE)

Research Organization:: Los Alamos National Laboratory (LANL), Los Alamos, NM (United States)

Sponsoring Organization:: USDOE National Nuclear Security Administration (NNSA)

Grant/Contract Number:: 89233218CNA000001

OSTI ID:: 2470431

Journal Information:: Neural Regeneration Research, Journal Name: Neural Regeneration Research Journal Issue: 11 Vol. 16; ISSN 1673-5374

Publisher:: Chinese Association of Rehabilitation Medicine (CARM)Copyright Statement

Country of Publication:: United States

Language:: English

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Longitudinal Evaluation of theHdh^(CAG)150Knock-In Murine Model of Huntington's Disease Heng, Mary Y.; Tallaksen-Greene, Sara J.; Detloff, Peter J. The Journal of Neuroscience, Vol. 27, Issue 34 https://doi.org/10.1523/JNEUROSCI.1830-07.2007	journal	August 2007
Cellular Localization of Huntingtin in Striatal and Cortical Neurons in Rats: Lack of Correlation with Neuronal Vulnerability in Huntington’s Disease Fusco, Francesca R.; Chen, Quan; Lamoreaux, William J. The Journal of Neuroscience, Vol. 19, Issue 4 https://doi.org/10.1523/JNEUROSCI.19-04-01189.1999	journal	February 1999
Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology Gutekunst, Claire-Anne; Li, Shi-Hua; Yi, Hong The Journal of Neuroscience, Vol. 19, Issue 7 https://doi.org/10.1523/JNEUROSCI.19-07-02522.1999	journal	April 1999
The Bifunctional microRNA miR-9/miR-9* Regulates REST and CoREST and Is Downregulated in Huntington's Disease Packer, Amy N.; Xing, Yi; Harper, Scott Q. The Journal of Neuroscience, Vol. 28, Issue 53 https://doi.org/10.1523/JNEUROSCI.2390-08.2008	journal	December 2008
Mitochondrial-Dependent Ca²⁺Handling in Huntington's Disease Striatal Cells: Effect of Histone Deacetylase Inhibitors Oliveira, Jorge M. A.; Chen, Sylvia; Almeida, Sandra The Journal of Neuroscience, Vol. 26, Issue 43 https://doi.org/10.1523/JNEUROSCI.3004-06.2006	journal	October 2006
Widespread Disruption of Repressor Element-1 Silencing Transcription Factor/Neuron-Restrictive Silencer Factor Occupancy at Its Target Genes in Huntington's Disease Zuccato, C.; Belyaev, N.; Conforti, P. Journal of Neuroscience, Vol. 27, Issue 26 https://doi.org/10.1523/JNEUROSCI.4278-06.2007	journal	June 2007
The use of the R6 transgenic mouse models of Huntington’s disease in attempts to develop novel therapeutic strategies Li, Jia Yi; Popovic, Natalija; Brundin, Patrik NeuroRX, Vol. 2, Issue 3 https://doi.org/10.1602/neurorx.2.3.447	journal	July 2005
Knock-in mouse models of Huntington’s disease Menalled, Liliana B. NeuroRX, Vol. 2, Issue 3 https://doi.org/10.1602/neurorx.2.3.465	journal	July 2005
Huntingtons Disease: New Frontiers for Molecular and Cell Therapy Melone, Mariarosa; Jori, Francesco; Peluso, Gianfranco Current Drug Targets, Vol. 6, Issue 1 https://doi.org/10.2174/1389450053344975	journal	February 2005
Regulatory Triangle of Neurodegeneration, Adult Neurogenesis and MicroRNAs Singh, Tanisha; Jauhari, Abhishek; Pandey, Ankita CNS & Neurological Disorders - Drug Targets, Vol. 13, Issue 1 https://doi.org/10.2174/18715273113126660190	journal	February 2014
Cellular Analysis of Silencing the Huntington’s Disease Gene Using AAV9 Mediated Delivery of Artificial Micro RNA into the Striatum of Q140/Q140 Mice Keeler, Allison M.; Sapp, Ellen; Chase, Kathryn Journal of Huntington's Disease, Vol. 5, Issue 3 https://doi.org/10.3233/JHD-160215	journal	October 2016
The involvement of microRNAs in neurodegenerative diseases Maciotta, Simona; Meregalli, Mirella; Torrente, Yvan Frontiers in Cellular Neuroscience, Vol. 7 https://doi.org/10.3389/fncel.2013.00265	journal	January 2013
Non-coding RNA interact to regulate neuronal development and function Iyengar, Bharat R.; Choudhary, Ashwani; Sarangdhar, Mayuresh A. Frontiers in Cellular Neuroscience, Vol. 8 https://doi.org/10.3389/fncel.2014.00047	journal	January 2014
The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for In vitro Studies on Huntingtin Function Szlachcic, Wojciech J.; Wiatr, Kalina; Trzeciak, Marta Frontiers in Molecular Neuroscience, Vol. 10 https://doi.org/10.3389/fnmol.2017.00253	journal	August 2017
MicroRNA-124 slows down the progression of Huntington′s disease by promoting neurogenesis in the striatum Kim, Manho; Liu, Tian; Im, Wooseok Neural Regeneration Research, Vol. 10, Issue 5 https://doi.org/10.4103/1673-5374.156978	journal	January 2015
A New Role for MicroRNA Pathways: Modulation of Degeneration Induced by Pathogenic Human Disease Proteins Bilen, Julide; Liu, Nan; Bonini, Nancy M. Cell Cycle, Vol. 5, Issue 24 https://doi.org/10.4161/cc.5.24.3579	journal	December 2006
Micro RNA -214,-150,-146a and-125b targetHuntingtingene Sinha, Mithun; Ghose, Jayeeta; Bhattarcharyya, Nitai P. RNA Biology, Vol. 8, Issue 6 https://doi.org/10.4161/rna.8.6.16035	journal	November 2011
miR-196a Enhances Neuronal Morphology through Suppressing RANBP10 to Provide Neuroprotection in Huntington's Disease Her, Lu-Shiun; Mao, Su-Han; Chang, Chih-Yi Theranostics, Vol. 7, Issue 9 https://doi.org/10.7150/thno.18813	journal	January 2017

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59 BASIC BIOLOGICAL SCIENCES
Huntington's disease
animal model
cell biology
cerebral cortex
huntingtin
microRNA
neurodegeneration
neurosciences & neurology
striatum
therapeutic strategies

Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

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