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Fig. 1 | Acta Neuropathologica Communications

Fig. 1

From: Minimal expression of dysferlin prevents development of dysferlinopathy in dysferlin exon 40a knockout mice

Fig. 1

Knock out of dysferlin exon 40a does not result in a dysferlinopathy phenotype. a Representative images of skeletal muscle sections from the psoas of 12-month-old dysferlin exon 40aKO mouse lines, BLAJ and WT mice stained with haematoxylin and eosin (to display overall morphology), wheat-germ agglutinin (WGA)/DAPI (to highlight central nuclei, indicated by white and dark arrow heads), collagen VI/DAPI (to evaluate fibrosis) and oil-red O (to evaluate lipid content). Skeletal muscle from all 40aKO mice appear relatively normal compared to BLAJ muscle, without the overt inflammation, variable fiber size, fatty replacement (asterisk) and excess fibrosis seen in BLAJ mice. b Analysis of centralised nuclei in WGA/DAPI stained sections demonstrates a gradient of centralised nuclei inversely related to dysferlin protein expression. c Quantitative analysis of the percentage of muscle tissue across a whole muscle tissue section shows a reduction in muscle tissue in BLAJs only. d Frequency distribution of muscle fiber diameter size across the six different lines, showing predominance of smaller sized fibers in BLAJ mice and similar sized fibers between WT and 40AKO mice. e Coefficient of variation in fiber diameter in each of the six different lines expressed as a percentage. BLAJ show the greatest fiber size variation compared to WT and 40aKO mice f Quantification of collagen VI-stained area per field of view (700 µm x 500 µm). There was no significant difference between WT and 40aKO mice. Collagen VI is only elevated in BLAJ mice g Oil-red O-stained areas were quantified across the entire muscle section using the Scanscope digital slide analysis software. Three readings were recorded and averaged per animal. No significant difference in lipid content was detected between WT and 40aKO mice, whereas BLAJs showed a trend for increased lipid content. In all graphs, each dot represents an individual animal, n = 3–4 in b-e and G, n = 4–6 in f. Data are expressed as mean ± SEM. Differences were tested by One-way ANOVA with Tukey’s multiple-comparisons test. *p < 0.05, **p < 0.01, ***p < 0.001

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