Figure 1.
The SOD-1 (G93A) transgene in the SOD-1 (G93A)PS line.
(A) Genotyping of the SOD-1 (G93A)PS mouse reveals a characteristic band from the SOD-1 (G93A) transgene. (B) Copy number of the SOD-1G93A transgene is reduced in the SOD-1 (G93A)PS mice by a factor of 6 when compared to normal SOD-1 (G93A) mice, littermates from the first SOD-1 (G93A)PS mouse or from newly rederived SOD-1 (G93A) mice (p<0.05).
Figure 2.
Weight loss and muscular strength deficit are delayed in the SOD-1 PS line.
(A) The SOD-1 (G93A)PS mice (n = 8) have a delayed onset of weight decrease when compared to the original line (n = 17) (p<0.05). (B) Normal SOD-1 Tg mice start with a decrease in muscle strength at the age of 70 days. SOD-1 (G93A)PS mice have normal muscular strength until the age of 310 days (p<0.05).
Figure 3.
Timed data for onset and survival.
Given are parameters for disease onset (A-C), and survival (D) for the SOD-1 (G93A) (n = 17) and SOD-1 (G93A)PS mice (n = 8). (A) Onset of weight decrease was defined as a drop of 5% of the mouse maximal weight. (B) First manifestation of symptoms that are linked to gait impairment in terms of limbs coordination and overall stance, and (C) time to first manifestation of paresis. (D) End point of the disease, defined as the inability of the mouse to right itself within 30 seconds.
Table 1.
Listed are key characteristics of ALS-like symptoms in the original SOD-1 (G93A) and in the new SOD-1 (G93A)PS line (mean±SEM).
Table 2.
Listed are survival times (mean±SEM) in the SOD-1 (G93A)PS line through 4 subsequent generations.