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@ARTICLE{Schmitz:164856,
      author       = {Schmitz, Matthias and Canaslan, Sezgi and Espinosa, Juan
                      Carlos and Fernández-Borges, Natalia and Villar-Piqué,
                      Anna and Llorens, Franc and Varges, Daniela and Maass,
                      Fabian and Torres, Juan Maria and Hermann, Peter and Zerr,
                      Inga},
      title        = {{V}alidation of {P}lasma and {CSF} {N}eurofilament {L}ight
                      {C}hain as an {E}arly {M}arker for {S}poradic
                      {C}reutzfeldt-{J}akob {D}isease.},
      journal      = {Molecular neurobiology},
      volume       = {59},
      number       = {9},
      issn         = {0893-7648},
      address      = {Totowa, NJ},
      publisher    = {Humana Press},
      reportid     = {DZNE-2022-01300},
      pages        = {1-9},
      year         = {2022},
      abstract     = {Biomarkers are becoming increasingly important for the
                      differential diagnosis of neurodegenerative diseases.
                      Previous observations indicated neurofilament light chain
                      (NfL) as a potential blood-based biomarker for sporadic
                      Creutzfeldt-Jakob disease (sCJD). Here, we investigated the
                      stability, inter-assay/intra-assay variation and the
                      regulation of NfL levels in CSF and plasma in a large cohort
                      of sCJD patients by using a single-molecule array (SIMOA).
                      We defined cutoffs for an accurate diagnosis and measured
                      plasma NfL level in prion-infected mice models at different
                      time points to identify the potential dynamics throughout
                      the disease. Our analyses confirmed CSF and plasma NfL as
                      stable and consistent marker for sCJD. Receiver operating
                      characteristic (ROC) curve analysis showed an AUC of
                      0.92-0.93 to distinguish sCJD from control groups. Newly
                      defined cutoffs revealed good diagnostic accuracies of CSF
                      and plasma NfL, indicated by a sensitivity of $80-83.5\%$
                      and a specificity of $87.4-91\%.$ Studies on two humanized
                      prion-infected mice lines (Tg340-PRNP 129MM and Tg361-PRNP
                      129VV) revealed increased plasma NfL levels in a late
                      pre-clinical or very early clinical stage between 120-150
                      days post-inoculation. In conclusion, our work supports the
                      potential use of CSF and plasma NfL as a very early
                      biomarker in sCJD diagnostic with good diagnostic
                      accuracies.},
      keywords     = {Animals / Biomarkers / Creutzfeldt-Jakob Syndrome:
                      diagnosis / Humans / Intermediate Filaments / Mice /
                      Neurofilament Proteins / Prions / tau Proteins / Biomarkers
                      (Other) / Diagnostic (Other) / Neurofilament light chain
                      (Other) / Plasma (Other) / Sporadic Creutzfeldt-Jakob
                      disease (Other)},
      cin          = {AG Zerr / Ext UMG Zerr / Clinical Dementia Research
                      Göttingen},
      ddc          = {570},
      cid          = {I:(DE-2719)1440011-1 / I:(DE-2719)5000037 /
                      I:(DE-2719)1440015},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:35716271},
      doi          = {10.1007/s12035-022-02891-7},
      url          = {https://pub.dzne.de/record/164856},
}