Figure 2.
PCC or CO treatment does not affect myelination directly, but exerts its effects on axons. (A) Semi-thin sections illustrate hypomyelination and the absence of large-diameter fibres in R98C/+ animals, and severe dysmyelination in R98C/R98C mice. Several large-diameter fibres can be observed in PCC or CO treated R98C/+ animals. Treatment failed to improve hypomyelination. Bar = 10 μm. (B) By 3 months of age, the distribution of axon diameter returned to normal in PCC or CO treated (not shown) R98C/+ mice (P < 0.001 for both CO and PCC when compared with untreated R98C/+); the proportion of large-diameter fibres was fully corrected (n = 2–3/genotype/treatment group). (C) Increased axonal density in untreated R98C/+ mice was markedly reduced in PCC (P < 0.001) or CO (P = 0.002) treated R98C/+ animals in correlation with the increase in axon size. * = P < 0.05; ** = P < 0.01; *** = P < 0.001.