Fig. 2
Intraperitoneal administration of hSOD1 aggregate seeds does not induce hSOD1 aggregation in the CNS or peripheral tissues. a and b Plots depict hSOD1 aggregates detected in the CNS (lumbar, thoracic, and cervical spinal cord, brainstem, brain and cerebellum) and peripheral organs (liver, kidney, sciatic nerve and quadriceps femoris muscle) of hSOD1G85R Tg mice that were inoculated with hSOD1G85R Tg or control spinal cord homogenates into the peritoneal cavity. Amounts of hSOD1 aggregates in tissue homogenates were analyzed with a binary epitope mapping assay using the hSOD1 57–72 Ra-Ab with an end-stage hSOD1G93A spinal cord homogenate used as standard (set to 1). a Black line indicates the median value. The shaded area indicates the range of background reactivity to spinal cord homogenates from non-Tg and SOD1-knockout mouse controls (mean ± 2 SD) (n = 8) [10]. Note that the antibody likely would show different background reactivities to dot-blotted homogenates of other tissues from controls. b Distribution of hSOD1 aggregates along the neuraxis in individual end-stage Tg mice. The amount of filtertrapped aggregates from different segments was normalized against the levels from the lumbar spinal cord. Note the altered scale used to visualize reactivity to brain and cerebellum homogenates. The results for individual mice are presented in different colors to improve distinction. c Immunohistopathological analysis of tissue sections from the lumbar spinal cord and brainstem of end-stage i.p. inoculated hSOD1G85R Tg mice. Tissue sections from the lumbar spinal cord and brainstem were stained using the hSOD1 131–153 Ra-Ab (green), isolectin B4 (red) as a marker for endothelial cells and DAPI (blue). Aggregated hSOD1 was similarly distributed around blood vessels in the lumbar spinal cord and brainstem in the end-stage mice inoculated with either hSOD1G85R Tg spinal cord homogenate or control homogenate. Scale bar represents 50 μm