Fig. 1.

Differential localization of intranuclear DPR inclusions in transduced primary neurons and in neurons of cases with C9orf72 mutation. Double immunofluorescence for different DPR proteins (green) and nucleolin (red), a marker for the nucleolus, in primary neurons (a) and in frontal cortex of cases with C9orf72 mutation (b). Nuclei are labeled with DAPI. Single confocal sections containing the nucleolus are shown. a Primary neurons transduced with lentivirus expressing either GFP-GR₁₄₉, PR₁₇₅-GFP, GA₁₇₅-GFP or GP₈₀-V5/His (DIV6 + 7). Note that poly-GR and poly-PR but not poly-GA intranuclear inclusions are localized in the nucleolus. Poly-GA forms mainly compact cytoplasmic inclusions. Poly-GP expression is mainly pan-nuclear and also cytosolic. b In cortical areas of cases with C9orf72 mutation neuronal intranuclear poly-GA, poly-GR and poly-GP inclusions are mostly localized adjacent to the nucleolus (red arrows) or less frequently randomly distributed (white arrows). No colocalization of DPR proteins with the nucleolus is observed. Scale bars represent 10 µm