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. 2014 Feb 18;111(9):3620–3625. doi: 10.1073/pnas.1312245111

Fig. 5.

Fig. 5.

Misfolded SOD1 is detectable in the spinal cords of both FALS and SALS patients. IHC using antibodies against misfolded SOD1 in cases of FALS with SOD1 mutations (A–D) and SALS (E and F). In cases with SOD1 mutations, neuronal cytoplasmic inclusions and neurites (arrow) were labeled in the primary motor cortex (A) and ventral gray matter of the spinal cord (B and C). The spinal gray matter (D) also contained numerous swollen axons. Immunopositive axons of normal caliber with a visible myelin sheath were present in the corticospinal tracts (arrows in E) and motor nerve roots (arrows in F) in sporadic cases. IHC was performed using 10E11C11 (A, B, and D–F) and 3H1 (C) antibodies. (Scale bars: 20 µm in A and D, 30 µm in B and C, and 12 µm in E and F.) (G) IP of 10% (wt/vol) spinal cord homogenate in PBS using DSE mAbs, normalized to equal concentrations of SOD1. Samples were preincubated at 37 °C for 30 min in the presence or absence of 200 µg/mL PK. All immunoblots were probed with pan-SOD1 pAb. C, cervical spinal cord; T, thoracic spinal cord.