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Fig. 3 | Orphanet Journal of Rare Diseases

Fig. 3

From: Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin

Fig. 3

VEP latency and amplitude in PN60, PN75, PN85 and PN100, either sham or HPßCD-treated, Npc1 +/+ and Npc1 −/− mice. a Histograms indicate the mean ± SEM of N1, N2, and N3 latency values obtained in different experimental groups at increasing ages, as indicated in the panel. Note that HPßCD fully rescued the latency increase observed in Npc1 −/− mice, whereas it had no effect on Npc1 +/+ mice. PN100 sham Npc1 −/− mice are not reported because these animals never survived beyond PN95, unless treated with HPßCD. b Effect of the age on N1 latency of various experimental group. Note that the latency significantly increased with age in sham Npc1 −/− mice, but not in Npc1 +/+ mice, and that such increase was effectively prevented by treatment with HPßCD (*, p < 0.05). c Histograms indicate the mean ± SEM of N1 amplitude values obtained in different experimental groups at increasing ages, as indicated in the panel. Asterisks indicate statistically significant differences among experimental groups (*, p < 0.05; **, p < 0.01; ***, p < 0.001)

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