Intramuscular viral delivery of paraplegin rescues peripheral axonopathy in a model of hereditary spastic paraplegia
J. Clin. Invest. Marinella Pirozzi, et al. 116:202 doi:10.1172/JCI26210 [
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Figure 6Intramuscular viral delivery of paraplegin improves the motor performance of
Spg7–/– mice.
(
A) Monthly performance of
Spg7+/+ mice, AAV-LacZ–treated
Spg7–/– mice, and AAV2/2-Spg7–treated
Spg7–/– mice on an accelerating rotarod apparatus beginning at 3 months, the time of the bilateral viral injection. The mice treated bilaterally with the AAV-Spg7 vector showed a progressive better performance compared with
Spg7–/– mice injected bilaterally with the AAV-LacZ vector. Where significant,
P values for individual Student’s
t test between the 2 groups of treated
Spg7–/– mice are shown. In addition, mice treated with AAV2/2-Spg7 displayed a statistically significant difference in their performance during the course of the experiment (1-way ANOVA,
P = 0.0001), as did the control mice (1-way ANOVA,
P = 0.008), in contrast to mice injected with AAV-LacZ (1-way ANOVA,
P = 0.07). (
B) Morphometric quantification of the percentage of affected axons in the sciatic nerves 10 months after treatment with AAV-LacZ or AAV2/2-Spg7 shows a statistically significant decrease due to the treatment. The
P value of Student’s
t test is shown.
