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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

From: Nebulin nemaline myopathy recapitulated in a compound heterozygous mouse model with both a missense and a nonsense mutation in Neb

Fig. 5

Whole-muscle physiology experiments showed the NebY2303H,Y935X EDL muscle was susceptible to eccentric contraction-induced damage. a, b Whole-muscle in vitro contractile analysis of extensor digitorum longus (EDL) and soleus (SOL) from male mice at 7 months of age showed a significant deficit in normalised force production in NebY2303H,Y935X at low-stimulation frequencies. c, d EDL NebY2303H,Y935X muscle was more susceptible to damage by eccentric contractions involving stretches to 120 to 140% of Lo than wild type. e, f Transient stretch-induced force responses were higher in NebY2303H,Y935X EDL muscles compared with wild type, suggesting increased stiffness in NebY2303H,Y935X EDL muscles. In contrast, transient stretch-induced force responses were lower in NebY2303H,Y935X SOL muscles compared with wild type, suggesting greater compliance in NebY2303H,Y935X SOL muscles. n = 7, Two-way ANOVA, *p < 0.05; **p < 0.005; #p < 0.0005; ¤p < 0.0001

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