Ighmbp2
D564N/D564N mice exhibit reduced muscle fiber size that is improved with ssAAV9-IGHMBP2 gene therapy. (A) Representative images of PND15 cross-sections of the gastrocnemius muscle from Ighmbp2+/+ mice, Ighmbp2D564N/D564N mice and Ighmbp2D564N/D564N ssAAV9-IGHMBP2 injected mice. Muscles were immunostained with anti-laminin antibody and images were taken at 40x magnification. Analysis of gastrocnemius muscle fiber area (B) and perimeter (C) from Ighmbp2+/+ mice (green), Ighmbp2D564N/D564N mice (red) and Ighmbp2D564N/D564N ssAAV9-IGHMBP2 injected mice (blue). Quantification of diaphragm muscle fiber area (D) and perimeter (E) from Ighmbp2+/+ mice (green), Ighmbp2D564N/D564N mice (red) and Ighmbp2D564N/D564N ssAAV9-IGHMBP2 injected mice (blue). Data bars expressed as mean ± SEM. ****P < 0.0001, ***P < 0.001, **P < 0.01, *P < 0.05, n = 3 animals per cohort (n > 200 muscle fibers were analyzed and averaged per cohort).