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. 2021 Nov 2;31(8):1293–1307. doi: 10.1093/hmg/ddab317

Figure 5.

Figure 5

Motor neuron number and size is reduced in Ighmbp2D564N/D564N mice and is partially rescued with ssAAV9-IGHMBP2 gene therapy. (A) Representative images of cross sections from PND15 L3–L5 spinal cord motor neurons of Ighmbp2+/+ mice (top panels), Ighmbp2D564N/D564N mice (middle panels) and Ighmbp2D564N/D564N ssAAV9-IGHMBP2 injected mice (bottom panels). Mice were injected with a high dose (5 × 1011 viral genomes) of ssAAV9-IGHMBP2. Sections are immunostained with anti-ChAT antibody and Nissl stain (Neurotrace). Fluorescent microscope images are taken at 40x magnification. (B-D) Motor neuron somas in Ighmbp2+/+ mice (green), Ighmbp2D564N/D564N mice (red) and Ighmbp2D564N/D564N ssAAV9-IGHMBP2 injected mice (blue). (B) Quantification of motor neuron soma number. (C) Area analysis of motor neuron somas. (D) Quantification of motor neuron soma perimeter. Data analyzed as a one-way ANOVA with Tukey’s post-hoc test for multiple comparisons. Data bars expressed as mean ± SEM. ****P < 0.0001, ***P < 0.001, **P < 0.01, *P < 0.05, n = 3 animals per cohort in which >400 cells were measured per cohort and averaged; ns = not significant.