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Appendix S1. Supplementary Materials and methods.

Figure S1. Human SOD1-immunoreactive inclusions. Human SOD1 inclusions in spinal ventral horns from an ALS patient and a transgenic mouse carrying the G127X mutation are here shown as examples. *denotes nuclei in motor neurons with abundant hSOD1 inclusions. The streaks of dots represent inclusions in axons and dendrites. The abundance of inclusions in the neuropil (area outside cell bodies: interstitium, neurites and glial processes) is generally greater in transgenic mice than in human ALS cases. The G127X patient was described in Jonsson et al. Ann. Neurol. 63, 2008, 675. The scale bars indicate 20 μm.

Figure S2. A spinal cord homogenate from a pre-symptomatic D90A (100 days) transgenic mouse was subjected to density-gradient ultracentrifugation. Mutant hSOD1 was detected by immunoblotting (IB). For detection of mutant hSOD1 aggregates, fractions were filter-trapped in a dot-blot apparatus. For discrimination between C57-C146 disulfide-oxidized (arrow) and reduced hSOD1 (arrowhead), reducing agent was omitted from the immunoblot sample buffer. SE = short exposure of the blot.

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