FIGURE

Fig. 8

ID
ZDB-FIG-210925-25
Publication
Riboldi et al., 2021 - Sumoylation regulates the assembly and activity of the SMN complex
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Fig. 8

SIM-less SMN fails to rescue sensory-motor circuit dysfunctions in SMA mice.

a Immunostaining of VGluT1 + synapses (white dots) and ChAT + MNs (blue) in L1 spinal cord sections from WT mice or SMA mice injected with AAV9-GFP, AAV9-SMN-WT or AAV9-SMN-2VA at P9. Scale bar = 20 µm; scale bar of the insert = 10 µm. b Representative traces of extracellular recordings from L1 ventral root following L1 dorsal root stimulation from WT mice or SMA mice injected with AAV9-GFP, AAV9-SMN-WT or AAV9-SMN-2VA at P9. Arrows indicate the maximum amplitude of the monosynaptic reflex. Arrowheads indicate the stimulus artifact. Scale bars = 0.5 mV and 10 ms. c Number of VGluT1 + synaptic boutons (white dots) on the somata of L1 MNs (blue) from the same groups as in (a) at P9. Data represent mean and SEM from WT, SMA + AAV9-GFP, SMA + AAV9-SMN-WT, and SMA + AAV9-SMN-2VA (average of boutons counted for at least 15 cells from 4 animals for each group, n = 4 animals). Statistical significance was determined by one-way ANOVA with Tukey’s post hoc test (adjusted P values: WT vs. SMA + GFP p < 0.0001; WT vs. SMA + SMN-WT p = 0.0085; WT vs. SMA + SMN-2VA p < 0.0001; SMA + GFP vs. SMA + SMN-WT p = 0.003; SMA + GFP vs. SMA + SMN-2VA p = 0.8208; SMA + SMN-WT vs. SMA + SMN-2VA p = 0.0132). d Quantification of spinal reflex amplitudes recorded from the same groups as in (b) at P9. Data represent mean and SEM (n = 4 animals). Statistical significance was determined by one-way ANOVA with Tukey’s post hoc test (adjusted P values: WT vs. SMA + GFP p = 0.0002; WT vs. SMA + SMN-WT p = 0.0633; WT vs. SMA + SMN-2VA p = 0.0002; SMA + GFP vs. SMA + SMN-WT p = 0.0254; SMA + GFP vs. SMA + SMN-2VA p = 0.9922; SMA + SMN-WT vs. SMA + SMN-2VA p = 0.0157). Significant differences with WT have been reported immediately atop of each bar plot, while comparisons between the other experimental conditions have been graphed with connecting lines.

Expression Data

Expression Detail
Antibody Labeling
Phenotype Data

Phenotype Detail
Acknowledgments
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