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@ARTICLE{Krau:141139,
      author       = {Krauß, Sybille and Nalavade, Rohit and Weber, Stephanie
                      and Carter, Katlynn and Evert, Bernd O},
      title        = {{U}pregulation of mi{R}-25 and mi{R}-181 {F}amily {M}embers
                      {C}orrelates with {R}educed {E}xpression of {ATXN}3 in
                      {L}ymphocytes from {SCA}3 {P}atients.},
      journal      = {MicroRNA},
      volume       = {8},
      number       = {1},
      issn         = {2211-5366},
      address      = {Sharjah [u.a.]},
      publisher    = {Bentham Science},
      reportid     = {DZNE-2020-07461},
      pages        = {76-85},
      year         = {2019},
      abstract     = {Spinocerebellar ataxia type 3 (SCA3), the most common
                      spinocerebellar ataxia, is caused by a polyglutamine (polyQ)
                      expansion in the protein ataxin-3 (ATXN3). Silencing the
                      expression of polyQ-expanded ATXN3 rescues the cellular
                      disease phenotype.This study investigated the differential
                      expression of microRNAs (miRNAs), small noncoding RNAs
                      targeting gene expression, in lymphoblastoid cells (LCs)
                      from SCA3 patients and the capability of identified
                      deregulated miRNAs to target and alter ATXN3
                      expression.MiRNA profiling was performed by microarray
                      hybridization of total RNA from control and SCA3-LCs. The
                      capability of the identified miRNAs and their target sites
                      to suppress ATXN3 expression was analyzed using mutagenesis,
                      reverse transcription PCR, immunoblotting, luciferase
                      reporter assays, mimics and precursors of the identified
                      miRNAs.SCA3-LCs showed significantly decreased expression
                      levels of ATXN3 and a significant upregulation of the
                      ATXN3-3'UTR targeting miRNAs, miR-32 and miR-181c and
                      closely related members of the miR-25 and miR-181 family,
                      respectively. MiR-32 and miR-181c effectively targeted the
                      3'UTR of ATXN3 and suppressed the expression of ATXN3.The
                      simultaneous upregulation of closely related miRNAs
                      targeting the 3'UTR of ATXN3 and the significantly reduced
                      ATXN3 expression levels in SCA3-LCs suggests that miR-25 and
                      miR-181 family members cooperatively bind to the 3'UTR to
                      suppress the expression of ATXN3. The findings further
                      suggest that the upregulation of miR-25 and miR-181 family
                      members in SCA3- LCs reflects a cell type-specific,
                      protective mechanism to diminish polyQ-mediated cytotoxic
                      effects. Thus, miRNA mimics of miR-25 and miR-181 family
                      members may prove useful for the treatment of SCA3.},
      keywords     = {3' Untranslated Regions / Ataxin-3: genetics / Ataxin-3:
                      metabolism / HeLa Cells / Humans / Lymphocytes: metabolism /
                      Machado-Joseph Disease: genetics / Machado-Joseph Disease:
                      metabolism / MicroRNAs: genetics / MicroRNAs: metabolism /
                      Repressor Proteins: genetics / Repressor Proteins:
                      metabolism / 3' Untranslated Regions (NLM Chemicals) /
                      MIRN25 microRNA, human (NLM Chemicals) / MIrn181 microRNA,
                      human (NLM Chemicals) / MicroRNAs (NLM Chemicals) /
                      Repressor Proteins (NLM Chemicals) / ATXN3 protein, human
                      (NLM Chemicals) / Ataxin-3 (NLM Chemicals)},
      cin          = {AG Krauß},
      ddc          = {570},
      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:30147021},
      doi          = {10.2174/2211536607666180821162403},
      url          = {https://pub.dzne.de/record/141139},
}