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@ARTICLE{Krauss:140673,
      author       = {Krauss, Sybille and Evert, Bernd O},
      title        = {{T}he {R}ole of {M}icro{RNA}s in {S}pinocerebellar {A}taxia
                      {T}ype 3.},
      journal      = {Journal of molecular biology},
      volume       = {431},
      number       = {9},
      issn         = {0022-2836},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DZNE-2020-06995},
      pages        = {1729-1742},
      year         = {2019},
      abstract     = {More than $90\%$ of the human genome are transcribed as
                      non-coding RNAs. While it is still under debate if all these
                      non-coding transcripts are functional, there is emerging
                      evidence that RNA has several important functions in
                      addition to coding for proteins. For example, microRNAs
                      (miRNAs) are important regulatory RNAs that control gene
                      expression in various biological processes and human
                      diseases. In spinocerebellar ataxia type 3 (SCA3), a
                      devastating neurodegenerative disease, miRNAs are involved
                      in the disease process at different levels, including the
                      deregulation of components of the general miRNA biogenesis
                      machinery, as well as in the cell type-specific control of
                      the expression of the SCA3 disease protein and other SCA3
                      disease-relevant proteins. However, it remains difficult to
                      predict whether these changes are a cause or a consequence
                      of the neurodegenerative process in SCA3. Further studies
                      using standardized procedures for the analysis of miRNA
                      expression and larger sample numbers are required to enhance
                      our understanding of the miRNA-mediated processes involved
                      in SCA3 disease and may enable the development of
                      miRNA-based therapeutics. In this review, we summarize the
                      findings of independent studies highlighting both the
                      disease-related and cytoprotective roles of miRNAs that have
                      been implicated so far in the disease process of SCA3.},
      subtyp        = {Review Article},
      keywords     = {Animals / Ataxin-3: genetics / Ataxin-3: metabolism /
                      Biomarkers: metabolism / Brain: metabolism / Brain:
                      pathology / Cell Line / Disease Models, Animal / Drosophila
                      melanogaster: genetics / Drosophila melanogaster: metabolism
                      / Gene Expression Regulation / Humans / Lymphocytes:
                      metabolism / Lymphocytes: pathology / Machado-Joseph
                      Disease: genetics / Machado-Joseph Disease: metabolism /
                      Machado-Joseph Disease: pathology / Machado-Joseph Disease:
                      therapy / Mice / MicroRNAs: genetics / MicroRNAs: metabolism
                      / Molecular Targeted Therapy: methods / Neurons: metabolism
                      / Neurons: pathology / Repressor Proteins: genetics /
                      Repressor Proteins: metabolism / Signal Transduction},
      cin          = {AG Krauß ; AG Krauß},
      ddc          = {610},
      cid          = {I:(DE-2719)1011006},
      pnm          = {342 - Disease Mechanisms and Model Systems (POF3-342)},
      pid          = {G:(DE-HGF)POF3-342},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:30664869},
      doi          = {10.1016/j.jmb.2019.01.019},
      url          = {https://pub.dzne.de/record/140673},
}