Table 2. Summary of lag times of SOD1 fibrillization for seeded reactions with pre-formed fibrils of SOD1 mutants into wtSOD1 protein and with spinal cord homogenates into G93A and wtSOD1 proteins.
Fibrillisation reaction | Fmax (a.u.) | T0.5 (hrs) | τ (hrs) | Lag time (hrs) |
Cross-seeded (CS) reactions with pre-formed SOD1 mutant fibril ( Figure 2 ) | ||||
G93A cs wtSOD1 | 22214±9547 | 54.6±0.6 | 10.9±0.4 | 32.8±0.3 |
G37R cs wtSOD1 | 6482±5934 | 54.6±2.4 | 8.5±2.2 | 37.6±2.0 |
A4V cs wtSOD1 | 9828±4252 | 49.7±2.0 | 8.3±1.8 | 33.2±1.7 |
G85R cs wtSOD1 | 26714±11056 | 172.9±1.0 | 18.1±0.7 | 136.7±0.5 |
Seeded reactions with spinal cord homogenates ( Figure 4 ) | ||||
Spinal cord tgSOD1G93A seeded into wtSOD1 protein | 2140±690 | 78.0±0.5 | 11.0±0.4 | 56.0±0.3 |
Spinal cord non- transgenic control seeded into wtSOD1 protein | 1352±114 | n/a | n/a | n/a |
Spinal cord tgSOD1wtSOD1 seeded into wtSOD1 protein | 1621±210 | n/a | n/a | n/a |
Spinal cord tgSOD1G93A seeded into G93A protein | 7469±4148 | 34.3±0.8 | 5.8±0.5 | 22.6±0.3 |
Spinal cord non- transgenic control seeded into G93A protein | 2702±444 | 111.1±1.9 | 15.0±1.4 | 81.1±0.9 |
Spinal cord tgSOD1wtSOD1 seeded into G93A protein | 2077±366 | 115.6±2.5 | 14.8±2.1 | 86.0±1.7 |
Fmax = average maximum fluorescence (arbitrary unit, a.u.), T0.5 = observed time at transition mid-point (hrs), lag time = incubation duration prior to initiation of fibrillization (hrs), τ = 1/propagation rate of fibril growth (hrs), tgSOD1G93A = transgenic SOD1 mice carrying the G93A mutation, tgSOD1wtSOD1 = transgenic wtSOD1 overexpressor mice; n/a = not available. Data shown are average values from 4 to 10 replicates (± SEM) pooled from 2 to 3 independent assays.