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. Author manuscript; available in PMC: 2019 Feb 6.
Published in final edited form as: Ann Neurol. 2018 Feb 6;83(2):269–282. doi: 10.1002/ana.25144

Figure 3.

Figure 3

(A, top) Typical equatorial reflection obtained from a fiber preparation of a NEM3 patient (P4). (A, bottom) The separation of the 1,0 equatorial reflections (which reflects thin-thick filament lattice spacing) is comparable between fibers of control subjects (n=3) and NEM3 patients (NEM3-P5, n=4) in an inactive state. (B) In an inactive state, the I1,1/I1,0 intensity ratio, reflecting the position of myosin-based cross-bridges relative to actin, was also not different between NEM3 patients and control subjects; (C) During activation, the expected increase in the I1,1/I1,0 intensity ratio was reduced in the NEM3 patients compared to control subjects.