Figure 3

Transverse spinal cord sections from an ALS Patient with SOD1 ^E100G mutation and sections from a control patient. (a) shows a SOD1 IHC, (b) a SOD1 PET-blot and (c) a protease treated SOD1-PET blot. In the IHC shown in more detail in d-e staining of neurons and glial cells is visible in the gray matter (arrows and arrow heads in d, respectively). (e) shows that glial cells in the white matter are also stained. (f) In the control spinal cord section SOD1 is only detected in neurons of the gray matter. In PET-blots SOD1 is labelled in the grey matter as well as in glial cells in the white matter (g). However, in PK treated PET-blots aggregates are strongly labelled in the grey matter area (h). Aggregates can only occasionally be seen in p62 IHC (i: ALS, j: control) and are undetectable in ubiquitin IHC (k: control, l: ALS). Bars represent 50 μm (a-f, h-l) and 100 μm (g).