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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

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

Fig. 4

NebY2303H,Y935X mice have significantly smaller myofibre diameters at 9 months of age. a-d MHC fibre types (I, IIA, IIB and mixed I/IIA and IIA/IIX) from 9-month-old female NebY2303H,Y935X extensor digitorum longus (EDL) and soleus (SOL) were counted and fibre diameters measured. a All myofibres except MHC type I were smaller in EDL from NebY2303H,Y935X mice compared with age matched wild-type littermates. b All fibres in SOL were smaller in NebY2303H,Y935X mice, although no mixed MHC type I/IIA were identified in this cohort. c, d Fibre-type proportions were measured in EDL (d, n = 3) and SOL (e, n = 3) but were not significantly different. Unpaired Mann-Whitney, *p < 0.05; **p < 0.005; ***p < 0.0005; ****p < 0.0001

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