title: Altered Expression of the Long Noncoding RNA NEAT1 in Huntington's Disease.
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.
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.|Department of Neurology, School of Medicine, Soonchunhyang University, Seoul, South Korea.
authors.forename: Jun-Sang
authors.initials: JS
authors.lastname: Sunwoo
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Soon-Tae
authors.initials: ST
authors.lastname: Lee
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Wooseok
authors.initials: W
authors.lastname: Im
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Mijung
authors.initials: M
authors.lastname: Lee
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.|Department of Neurology, Kyung Hee University Hospital at Gangdong, Seoul, South Korea.
authors.forename: Jung-Ick
authors.initials: JI
authors.lastname: Byun
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Keun-Hwa
authors.initials: KH
authors.lastname: Jung
authors.affiliation: Department of Neurology, Healthcare System Gangnam Center, Seoul National University Hospital, Seoul, South Korea.
authors.forename: Kyung-Il
authors.initials: KI
authors.lastname: Park
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Ki-Young
authors.initials: KY
authors.lastname: Jung
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea.
authors.forename: Sang Kun
authors.initials: SK
authors.lastname: Lee
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. stemcell.snu@gmail.com.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea. stemcell.snu@gmail.com.
authors.forename: Kon
authors.initials: K
authors.lastname: Chu
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. kimmanho@snu.ac.kr.|Program in Neuroscience, College of Medicine, Seoul National University, Seoul, South Korea. kimmanho@snu.ac.kr.|Protein Metabolism Medical Research Center, College of Medicine, Seoul National University, Seoul, South Korea. kimmanho@snu.ac.kr.
authors.forename: Manho
authors.initials: M
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D045744:Cell Line, Tumor; D005260:Female; D015870:Gene Expression; D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008808:Mice, Inbred CBA; D008822:Mice, Transgenic; D008875:Middle Aged; D062085:RNA, Long Noncoding / Q000096:biosynthesis* / Q000235:genetics*
chemical_list: C577276:NEAT1 long non-coding RNA, human; D062085:RNA, Long Noncoding
keywords: Huntington’s disease; Long noncoding RNA NEAT1; Microarray; Neuroprotection