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Fig. 1 | Acta Neuropathologica Communications

Fig. 1

From: NEB mutations disrupt the super-relaxed state of myosin and remodel the muscle metabolic proteome in nemaline myopathy

Fig. 1

Myosin structure and relaxed conformational states in humans. Typical Mant-ATP chase experimental data show exponential decays for muscle fibres isolated from all the different groups (A). The proportion of myosin molecules in the disordered-relaxed (P1, B) and super-relaxed states (P2, C) as well as their respective ATP turnover lifetimes (T1, D and T2, E) are presented. F Shows the P1 data as a function of age. By staining and imaging myosin using super-resolution microscopy (G, scale bar = 10 µm) myosin filament length is calculated (H). Dots are individual subject’s average data. Means and standard deviations also appear on histograms. *Denotes a difference (p < 0.05) when compared with controls (CTL)

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