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Molecular Neurobiology

, Volume 54, Issue 2, pp 1577–1586 | Cite as

Altered Expression of the Long Noncoding RNA NEAT1 in Huntington’s Disease

  • Jun-Sang Sunwoo
  • Soon-Tae Lee
  • Wooseok Im
  • Mijung Lee
  • Jung-Ick Byun
  • Keun-Hwa Jung
  • Kyung-Il Park
  • Ki-Young Jung
  • Sang Kun Lee
  • Kon Chu
  • Manho Kim
Article

Abstract

Huntington’s disease (HD) is a devastating neurodegenerative disease caused by cytosine-adenine-guanine trinucleotide repeat expansion in the huntingtin gene. Growing evidence supports the regulatory functions of long noncoding RNAs (lncRNAs) in the disease process, but little is known about the association between lncRNAs and neuronal death in HD. Here, we evaluated the altered expression profiles of lncRNA in HD by using microarrays. Among dysregulated lncRNAs, we focused on the upregulation of nuclear paraspeckle assembly transcript 1 (NEAT1). Quantitative PCR analysis validated increased NEAT1 levels in the R6/2 mouse brain as well as the human HD postmortem brain. To determine the biological effects of NEAT1 on neuronal survival, neuro2A cells were transfected with the NEAT1 short isoform vector and were subjected to H2O2-induced injury. Subsequently, NEAT1-transfected cells showed increased viability under oxidative stress. Our observations support the notion that NEAT1 upregulation in HD contributes to the neuroprotective mechanism against neuronal injury rather than the pathological process underlying neurodegeneration in HD.

Keywords

Huntington’s disease Long noncoding RNA NEAT1 Microarray Neuroprotection 

Notes

Acknowledgments

This study was supported by grants from the Korean Health Technology R&D Project (HI14C2348), Ministry of Health and Welfare, Republic of Korea, and the National Research Foundation (NRF) of Korea (2011–0012728). S-T.L was supported by the Seoul National University Hospital Research Fund (04-2014-0730) and Korean Health Technology R&D Project (HI12C1773).

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Funding

This study was supported by grants from the Korean Health Technology R&D Project (HI14C2348), Ministry of Health and Welfare, Republic of Korea, and the National Research Foundation (NRF) of Korea (2011–0012728). S-T.L was supported by the Seoul National University Hospital Research Fund (04-2014-0730) and Korean Health Technology R&D Project (HI12C1773).

Supplementary material

12035_2016_9928_MOESM1_ESM.docx (32 kb)
ESM 1 (DOCX 32 kb)

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Jun-Sang Sunwoo
    • 1
    • 2
    • 3
  • Soon-Tae Lee
    • 1
    • 2
  • Wooseok Im
    • 1
    • 2
  • Mijung Lee
    • 1
    • 2
  • Jung-Ick Byun
    • 1
    • 2
    • 4
  • Keun-Hwa Jung
    • 1
    • 2
  • Kyung-Il Park
    • 5
  • Ki-Young Jung
    • 1
    • 2
  • Sang Kun Lee
    • 1
    • 2
  • Kon Chu
    • 1
    • 2
  • Manho Kim
    • 1
    • 2
    • 6
  1. 1.Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research InstituteSeoul National University HospitalSeoulSouth Korea
  2. 2.Program in Neuroscience, College of MedicineSeoul National UniversitySeoulSouth Korea
  3. 3.Department of Neurology, School of MedicineSoonchunhyang UniversitySeoulSouth Korea
  4. 4.Department of NeurologyKyung Hee University Hospital at GangdongSeoulSouth Korea
  5. 5.Department of Neurology, Healthcare System Gangnam CenterSeoul National University HospitalSeoulSouth Korea
  6. 6.Protein Metabolism Medical Research Center, College of MedicineSeoul National UniversitySeoulSouth Korea

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