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. 2012 Dec 14;135(12):3551–3566. doi: 10.1093/brain/aws299

Figure 3.

Figure 3

PCC or CO treatment improves the innervation of neuromuscular junctions in R98C animals. (A) Magnified (×60) confocal images of neuromuscular junctions of wild-type and R98C animals at 6 weeks of age. Arrows indicate the demyelinated segment. Scale bar = 10 μm. (B and C) Virtually all neuromuscular junctions of untreated and PCC/CO treated wild-type mice were fully myelinated. Untreated wild-type values are included as control in the graphs. Percentage of fully myelinated neuromuscular junctions in the triangularis sterni muscle in R98C mice at post-natal Day 13, 6 weeks and 3 months of age. A tendency towards a lower number of normally myelinated junctions could be seen at post-natal Day 13 in R98C mice. By 6 weeks of age, R98C/R98C animals exhibited a significantly reduced number of normally myelinated junctions, which was rescued by CO treatment (P = 0.004). Benefits of treatment in R98C/R98C animals could be maintained even at 3 months of age (PCC P = 0.013, CO P = 0.017). Virtually all neuromuscular junctions of wild-type mice were fully myelinated, data not shown in the graphs. (C) The length of demyelinated segments proved to be longer in R98C/R98C animals than in R98C/+ mice (post-natal Day 13 P < 0.001, 6 week P = 0.003). CO treatment resulted in shorter demyelinated segments in R98C/R98C animals (post-natal Day 13 P < 0.001, 3 month P = 0.00815). NMJ = neuromuscular junction.