Figure 1.

Western blot analysis with NPC1-C antibody of normal human fibroblasts (C), fibroblasts from an NP-C patient (NP-C), CHO cells from the mutant cell line CT60 that display an NP-C phenotype (CT60), and CT60 cells transfected with yeast artificial chromosome 911D5 containing the NPC1 gene (D5B5). Normal fibroblasts express NPC1 as a 165 kDa protein that is absent in NP-C fibroblasts and in CT60 CHO cells. A slightly larger sized NPC1 band is observed in D5B5 cells probably because of differential posttranslational processing of NPC1 in CHO cells. The NPC1 immunoreactive band is completely abolished by preincubation with antibody absorbed with NPC1-C peptide. Representative of three separate experiments.