Fig. 1

Changes in SVZ morphology in patients with ALS. a-b Illustrations show increases in thickness of the gap and ribbon layers; increases were greater in patients with ALS-FTD. c Increased expression of GFAP in the gap and ribbon layers in patients with ALS and ALS-FTD. d Images from the semithin sections of the SVZ showing the ventricular wall (V), the ependymal monolayer (I), the gap layer (II), the ribbon layer (III) and the parenchyma (IV). Data from these images was used to prepare the graphics in A and B. e-f Quantitative graphics representing the proliferative activity in the SVZ of ALS patients based on the analysis of the cell proliferation markers PCNA and Ki-67. PCNA and Ki-67 showed significant increases in PCNA and Ki-67 marking. g Confocal microscopy images of the SVZ in controls and patients with ALS and ALS-FTD showing increased Ki-67 and GFAP marking in patients, arrows show a immunopositive cells. h Images of SVZ of Control and ALS/FTD Patients. IN ALS/FTD, a displayed GFAP positive projections in contact with the ventricle (arrows). In control samples no were observed. i Graph displaying expression of GFAPδ in the SVZ of healthy individuals and patients with ALS and ALS-FTD; expression is significantly higher in patients. j-k Graphs showing increased expression of the proteins occurring in neurogenesis and neuronal migration in the adult central nervous system (doublecortin, PSA-NCAM); protein levels were significantly higher in patients with ALS or ALS-FTD than in healthy controls. l Immunofluorescence study of GFAPδ expression showing increased levels in the SVZ in ALS or ALS-FTD patients. In the details it is observed that in patients greater immunohitochemistry marking shown. m Images showing cells positive for doublecortin near the lateral wall. Their elongated fusiform shape is typical of migrating cells (arrows). n Illustration of typical SVZ morphology from controls and ALS patients, showing the main changes we observed: thickening of the gap and ribbon layers with increased expression of markers of astrocytes, cell proliferation, and neurogenesis. Graphs show the mean + standard error (*p < 0.05; **p < 0.01). Scale bars: D: 50 μm; G-H, L and M: 15 μm