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. 2023 Jul 6;24(8):e56525. doi: 10.15252/embr.202256525

Figure 5. Intrathecal hEN1 injection in En1‐Het mice restores strength and prevents αMN death.

Figure 5

  1. Mice were tested for strength at 3 months of age, before the onset of αMN loss, but when strength has already decreased in En1‐het mice as measured in the forepaw grip strength, inverted grid, and extensor reflex tests (left side of each graph). The next day, the En1‐het mice were separated into two groups. One group received buffer and the other group recombinant hEN1 (1 μg in 5 μl), injected at the L5 level. One and a half months later (4.5 months of age) En1‐het mice injected with hEN1 have recovered normal strength, in contrast with noninjected mice or mice injected with buffer. Unpaired two‐sided t‐test used at 3 months of age. ****P < 0.0001. For 4.5‐month comparisons, 1‐way ANOVA followed by Tukey corrected post hoc comparisons. ***P < 0.0005; ****P < 0.0001. n = 9–31.
  2. At 4.5 months, following hEN1 injection at 3 months, the percentage of fully occupied endplates and the number of αMNs are not significantly different from control values. 1‐way ANOVA followed by Tukey corrected post hoc comparisons. ***P < 0.0005; ****P < 0.0001. n = 5–21.

Data information: The weakness and reversal with hEN1 and the neuroprotection were replicated in five independent experiments. Values are mean ± SD.

Source data are available online for this figure.