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Expanded GGGGCC (G4C2) nucleotide repeats within the C9ORF72 gene are the most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Sense and antisense transcripts of these expansions are translated to form five dipeptide repeat proteins (DRPs). We employed primary cortical and motor neuron(More)
The reprogramming of somatic cells to pluripotency using defined transcription factors holds great promise for biomedicine. However, human reprogramming remains inefficient and relies either on the use of the potentially dangerous oncogenes KLF4 and CMYC or the genetic inhibition of the tumor suppressor gene p53. We hypothesized that inhibition of signal(More)
 Figure S1 (related to Figure 1) shows the randomized codon sequences encoding the different DRPs. Modules of DRP-encoding specific 25-long repeats are shown. The modules are repeated in the construct to achieve the desired DRP length.  Figure S2 (related to Figure 1) shows validation of DRP constructs, expression analysis and localization of di-peptide(More)
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