Fig. 3.

Differential colocalization of DPR and p62 inclusions in cases with C9orf72 mutation and cell culture. Double immunofluorescence for DPR proteins and p62. Nuclei are labeled with DAPI. a In primary hippocampal neurons transduced with GA₁₇₅-GFP, GFP-GR₁₄₉ or GP₈₀-V5/His (DIV6 + 7) p62 co-aggregates with cytoplasmic and intranuclear poly-GA inclusions, but not with poly-GR and poly-GP. In contrast to poly-GA inclusions, poly-GR and poly-GP aggregates appear less compact or granular. b In frontal cortex of C9orf72 mutation patients almost all poly-GA and poly-GR and all poly-GP inclusions are positive for p62 (orange arrows). Poly-GA and poly-GR inclusions without p62 labeling are rare (white arrows). c Intranuclear p62 aggregates show the same distribution pattern as DPR inclusions and are mostly para-nucleolar (arrow). Scale bars represent 20 µm