Figure 7. Selective reduction of mutant SOD1 expression within endothelial cells by Cre-mediated gene excision does not affect ALS-like disease onset, progression, or survival.

(A) Relative mRNA abundance of SOD1^G93A in laser-captured microvessels and motor neurons in SOD1^G93A mice. n = 5. (B) Excision of the SOD1^G37R transgene in microvessels purified from SOD1^G37R mice or Ve-Cre/SOD1^G37R mice promoted Cre recombinase transgene. DNA copy number for the mutant SOD1 gene was normalized to mouse apoB gene. From 2–3 mice per extraction, 3 or 4 preparations of vessel-extracted DNA were analyzed. Excision frequency was corrected for the known 4:1 ratio of endothelial cells/pericytes. Data are mean ± SD. n = 3–4. (C–E) Age of disease onset (C), progression to an early phenotypic stage (D), and survival (E) in SOD1^G37R and Ve-Cre/SOD1^G37R mice. (F) IgG signal intensity in the lumbar spinal cords in SOD1^G37R mice (n = 4) and Ve-Cre/SOD1^G37R mice (n = 4) at disease onset at 7 months of age compared with age-matched nontransgenic littermate controls (n = 3).