Based on this approach, researchers have developed different disease models using either RNA interference in human neuroblastoma cells (Bolinches-Amoros et al., 2014; Palomo et al., 2011) or Caenorhabditis elegans (Vazquez-Manrique et al., 2006), or expressing a reduced amount of human FXN in the humanized mouse model YG8R (Al-Mahdawi et al., 2006). Here, FXN is linked to neuroblastoma.