Cerebellar lncRNA Expression Profile Analysis of SCA3/MJD Mice

Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) is the most common autosomal dominant spinocerebellar ataxia in China with highly clinical heterogeneity, such as progressive cerebellar ataxia, dysarthria, pyramidal signs, external ophthalmoplegia, dysphagia, and distal muscle at...

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Bibliographic Details
Published in:International journal of genomics 2018-01, Vol.2018 (2018), p.1-6
Main Authors: Xie, Yue, He, Lang, Peng, Huirong, Qiu, Rong, Xia, Kun, Tang, Bei-sha, Jiang, Hong, Wang, Puzhi, Yuan, Hongyu, Hou, Xiaocan, Wang, Chunrong, Peng, Yun, Chen, Zhao, Li, Tianjiao, Long, Zhe, Zhou, Xin
Format: Article
Language:English
Subjects:
Artificial chromosomes
Ataxia
Atrophy
Capillary electrophoresis
Cerebellar ataxia
Cerebellum
Deoxyribonucleic acid
Disease
DNA
Dysphagia
Gene expression
Genotype & phenotype
Huntington's disease
Machado-Joseph disease
Neurobiology
Neurosciences
Ophthalmoplegia
Pathogenesis
Physiology
Polyglutamine
Proteins
RNA polymerase
Spinocerebellar ataxia
Trinucleotide repeats
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Summary:Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) is the most common autosomal dominant spinocerebellar ataxia in China with highly clinical heterogeneity, such as progressive cerebellar ataxia, dysarthria, pyramidal signs, external ophthalmoplegia, dysphagia, and distal muscle atrophy. It is caused by the abnormal expansion of CAG repeats in a coding region of ATXN3. However, by focusing on the ATXN3 itself cannot fully explain the heterogeneous clinical features of SCA3/MJD. With the discovery of the increasing number of long noncoding RNAs (lncRNAs) that are believed to be involved in spinocerebellar ataxia type 8 (SCA8) and Huntington disease (HD), we wonder whether the lncRNAs are differentially expressed in the SCA3/MJD patients compared to the nonpatients. As the first step, we used lncRNA-Seq to investigate differential expression of the lncRNAs in the SCA3/MJD mice. Two known lncRNAs, n297609 and n297477, and a novel lncRNA TCONS_00072962 have been identified in SCA3/MJD mice with abnormal expression. The first discovery of the novel lncRNA TCONS_00072962 enriched the lncRNA expression profile in the SCA3/MJD mouse model.
ISSN:2314-436X
2314-4378
DOI:10.1155/2018/5383517