Figure 1From: Deleterious effects of lymphocytes at the early stage of neurodegeneration in an animal model of amyotrophic lateral sclerosis Delayed clinical onset in male mutant SOD1 transgenic mice deficient in the RAG2 gene. (A) Disease progression was significantly delayed in mSOD1/RAG2-/- mice (filled circles; n = 11) compared with mSOD1/RAG2+/+ mice (open circles; n = 13). (B) Kaplan Meier curve for symptomatic onset in mSOD1/RAG2+/+ mice (n = 13) and mSOD1/RAG2-/- mice (n = 11). Mice were evaluated for signs of motor deficiency with the following 4-point scoring system: 4 points, normal (no sign of motor dysfunction); 3 points, hind limb tremors were evident when suspended by the tail; 2 points, gait abnormalities were present; 1 point, dragging of at least one hind limb; 0 points, inability to right self within 30 s. We defined the point when the clinical score became 3 as being clinical onset. The mSOD1/RAG2-/- mice exhibited the disease phenotype at a later stage than the mSOD1/RAG2+/+ mice. (C) Time course of motor performance in the hanging-wire test. mSOD1/RAG2-/- mice (n = 11) performed significantly better than mSOD1/RAG2+/+ mice (n = 13). (D) Changes in mean body weight of mSOD1/RAG2-/- mice (n = 11) and mSOD1/RAG2+/+ mice (n = 13). (E) Kaplan-Meier survival curve. Absence of T and B cells did not affect the survival of mSOD1 mice (mSOD1/RAG2+/+ mice; n = 13, mSOD1/RAG2-/- mice; n = 11) (p = 0.13). Data are expressed as the mean ± SEM. Statistical analysis was done using the generalized Wilcoxon test for Kaplan-Meier curves (B,E) and a two-way repeated multivariate ANOVA (A, C, D) during 12 to 16 weeks of age. *:p < 0.05, #:p < 0.005.Back to article page