Correlation of age of onset and clinical severity in Niemann-Pick disease type C1 with lysosomal abnormalities and gene expression.

Pairwise analysis of the LysoTracker levels for the 24 patient cell lines included in both studies showed a highly significant correlation with previous measurements (Spearman r = 0.76, p < 0.0001, Fig. 1 ), demonstrating the reproducibility of LysoTracker staining in cultured primary fibroblasts from individuals with NPC1.

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