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

Figure 3.

Figure 3

Ighmbp2 D564N/D564N mice exhibit hindlimb atrophy and reduced motor function that can be improved by ssAAV9-IGHMBP2 gene therapy. (A) Representative images of PND7 and (B) PND16 wild-type and D564N mutant mice performing the HLS assay. Bar indicates the distance measured as an evaluation of relative splay. (C) Motor performance was analyzed using the TTR test in seconds (one-way ANOVA P < 0.0001). TTR from PND8 to PND18 in wild-type (n = 8), +/D564N (n = 8), D564N/D564N (n = 8) and D564N/D564N mice ICV injected with a high dose (5 × 1011 viral genomes) of ssAAV9-IGHMBP2 (n = 6). (D) HLS in D564N mutant mice ICV injected with a high dose (5 × 1011 viral genomes) of ssAAV9-IGHMBP2 along with wild type and D564N/D564N animals. (E) Motor strength and coordination assayed by rotarod in wild-type and D564N/D564Nmice ICV injected with a high dose (5 × 1011 viral genomes) of ssAAV9-IGHMBP2 (n = 14). Assay is measured in seconds. (F) Grip strength in wild type and D564N/D564N mice ICV injected with a high dose (5 × 1011 viral genomes) of ssAAV9-IGHMBP2 (n = 14). Data bars expressed as mean ± SEM; *P < 0.05, **P < 0.01, * indicates significance.