Fig. 2

Cytoplasmic incorporation of FUS is present in ALS-FUS patient brain and spinal cords. a DAB staining depicts cytoplasmic neuronal inclusions of FUS (as indicated by their morphology) in the precentral gyrus of FUS (p.G504Wfs*12) patient (bottom) compared to the nucleus staining of FUS in a normal control (CTL 4, top) and a sporadic ALS patient (middle). Prominent cytoplasmic or decreased nucleus staining of FUS with ring-like perinuclear inclusions were observed in the motor neurons of the ALS patient. The enlarged images are shown in the right panels. Scale bars = 10 μm. b FUS pathology was confirmed by double-label immunofluorescence for FUS (green) and NeuN (red) in a normal control (top), sporadic ALS patient (middle), and FUS (p.G504Wfs*12) patient (bottom). Boxed region in the left panel is enlarged in the right panels. Note that cytoplasmic FUS expressed in a normal control are microglia (Additional file 2: Figure S2). Cells were counter stained with the nuclear marker DAPI (blue). Scale bars = 50 μm for the merged left panels and 7.5 μm for the right panels. c The ventral horn of the cervical spinal cord sections from normal control (top), sporadic ALS patient (top), and FUS (p.G504Wfs*12) patient (bottom) were compared. The same pathological features were observed by DAB staining in the spinal cords of the FUS (p.G504Wfs*12) patient. Scale bars = 10 μm. d The corresponding sections were processed for double-label immunofluorescence. FUS pathology was confirmed by FUS (green) and NeuN (red) staining. Cells were counter stained with the nuclear marker DAPI (blue). Scale bars = 10 μm