Induction of myasthenia by immunization against muscle-specific kinase
J. Clin. Invest. Kazuhiro Shigemoto, et al. 116:1016 doi:10.1172/JCI21545 [
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Figure 1Rabbits manifest MG-like paresis after immunization with MuSK protein. (
A) Two rabbits, representative of the 4 animals that showed myasthenia, manifested myasthenic weakness after immunization with the recombinant MuSK protein. After 3 injections of MuSK protein, M1 and M2 rabbits manifested flaccid weakness within 3 and 9 weeks, respectively. The M2 rabbit developed severe exhaustion with muscle weakness. (
B) Cross sections from the soleus muscles of 2 paretic (M1 and M2) and 1 normal rabbit were stained with H&E. Muscle fibers in the M1 paretic rabbit showed only subtle changes in shape and smallness, whereas an atrophy of muscle fibers in the M2 paretic rabbit was observed as small angular fibers (arrows). Scale bar: 50 μm. (
C) Electromyograms recorded from the M1 paretic rabbit. The retroauricular branch of the facial nerve was continuously stimulated by a constant-current stimulator, which delivered square-wave pulses of 0.1 ms at 20 Hz, and the compound muscle action potential (the second peak observed on the oscilloscope screen was recorded at the indicated time points during stimulation) showed a decremental pattern, consistent with MG.