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@ARTICLE{Llorens:139770,
      author       = {Llorens, Franc and Thüne, Katrin and Martí, Eulàlia and
                      Kanata, Eirini and Dafou, Dimitra and Díaz-Lucena, Daniela
                      and Vivancos, Ana and Shomroni, Orr and Zafar, Saima and
                      Schmitz, Matthias and Michel, Uwe and Fernández-Borges,
                      Natalia and Andréoletti, Olivier and Del Río, José
                      Antonio and Díez, Juana and Fischer, Andre and Bonn, Stefan
                      and Sklaviadis, Theodoros and Torres, Juan Maria and Ferrer,
                      Isidre and Zerr, Inga},
      title        = {{R}egional and subtype-dependent mi{RNA} signatures in
                      sporadic {C}reutzfeldt-{J}akob disease are accompanied by
                      alterations in mi{RNA} silencing machinery and biogenesis.},
      journal      = {PLoS pathogens},
      volume       = {14},
      number       = {1},
      issn         = {1553-7374},
      address      = {Lawrence, Kan.},
      publisher    = {PLoS},
      reportid     = {DZNE-2020-06092},
      pages        = {e1006802},
      year         = {2018},
      abstract     = {Increasing evidence indicates that microRNAs (miRNAs) are
                      contributing factors to neurodegeneration. Alterations in
                      miRNA signatures have been reported in several
                      neurodegenerative dementias, but data in prion diseases are
                      restricted to ex vivo and animal models. The present study
                      identified significant miRNA expression pattern alterations
                      in the frontal cortex and cerebellum of sporadic
                      Creutzfeldt-Jakob disease (sCJD) patients. These changes
                      display a highly regional and disease subtype-dependent
                      regulation that correlates with brain pathology. We
                      demonstrate that selected miRNAs are enriched in sCJD
                      isolated Argonaute(Ago)-binding complexes in disease,
                      indicating their incorporation into RNA-induced silencing
                      complexes, and further suggesting their contribution to
                      disease-associated gene expression changes. Alterations in
                      the miRNA-mRNA regulatory machinery and perturbed levels of
                      miRNA biogenesis key components in sCJD brain samples
                      reported here further implicate miRNAs in sCJD gene
                      expression (de)regulation. We also show that a subset of
                      sCJD-altered miRNAs are commonly changed in Alzheimer's
                      disease, dementia with Lewy bodies and fatal familial
                      insomnia, suggesting potential common mechanisms underlying
                      these neurodegenerative processes. Additionally, we report
                      no correlation between brain and cerebrospinal fluid (CSF)
                      miRNA-profiles in sCJD, indicating that CSF-miRNA profiles
                      do not faithfully mirror miRNA alterations detected in brain
                      tissue of human prion diseases. Finally, utilizing a sCJD
                      MM1 mouse model, we analyzed the miRNA deregulation patterns
                      observed in sCJD in a temporal manner. While fourteen
                      sCJD-related miRNAs were validated at clinical stages, only
                      two of those were changed at early symptomatic phase,
                      suggesting that the miRNAs altered in sCJD may contribute to
                      later pathogenic processes. Altogether, the present work
                      identifies alterations in the miRNA network, biogenesis and
                      miRNA-mRNA silencing machinery in sCJD, whereby
                      contributions to disease mechanisms deserve further
                      investigation.},
      keywords     = {Adult / Aged / Aged, 80 and over / Brain: metabolism /
                      Brain: pathology / Case-Control Studies / Creutzfeldt-Jakob
                      Syndrome: classification / Creutzfeldt-Jakob Syndrome:
                      genetics / Creutzfeldt-Jakob Syndrome: pathology / Female /
                      Gene Expression Profiling / Humans / Male / MicroRNAs:
                      biosynthesis / MicroRNAs: genetics / Middle Aged / RNA
                      Interference / Transcriptome / MicroRNAs (NLM Chemicals)},
      cin          = {AG Zerr / Ext UMG Zerr / AG Bonn 1 / AG Fischer 1},
      ddc          = {610},
      cid          = {I:(DE-2719)1440011-1 / I:(DE-2719)5000037 /
                      I:(DE-2719)1410003 / I:(DE-2719)1410002},
      pnm          = {344 - Clinical and Health Care Research (POF3-344) / 342 -
                      Disease Mechanisms and Model Systems (POF3-342)},
      pid          = {G:(DE-HGF)POF3-344 / G:(DE-HGF)POF3-342},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:29357384},
      pmc          = {pmc:PMC5794191},
      doi          = {10.1371/journal.ppat.1006802},
      url          = {https://pub.dzne.de/record/139770},
}