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Figure 3. Structural observations

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1 Figure 3. Structural observations
Structural observations (A) ATPase reaction after preincubation at pH 9.4 reveals type 2 fiber atrophy and type 1 fiber hypertrophy. (B) Cytochemical reaction for AChE in longitudinally oriented fibers shows pretzel-shaped or linearly arrayed EP regions. (C and D) Colocalization of AChE (green signal) and the AChR (red signal) in cryosections of transversely oriented fibers reveals normal expression of both proteins at the EPs. (E) Electron micrograph depicts a normal EP region. Inset in (E) shows docking of synaptic vesicles at an active zone marked by a thickened segment of the presynaptic membrane. (F) Localization of AChR with peroxidase-labeled α-bungarotoxin. The black reaction product for AChR is normally distributed on terminal expansions of the junctional folds. Bars: (A, B): 50 μm; (C, D): 25 μm; (E, F): 0.5 μm; and inset in (E): 0.1 μm. AChE = acetylcholinesterase; AChR = acetylcholine receptor; AZ = active zone; EP = endplate; JF = junctional fold. Andrew G. Engel et al. Neurol Genet 2016;2:e105 © 2016 American Academy of Neurology

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