Fig. 7.

Correction of cholesterol transport in Niemann-Pick type C (NPC) fibroblasts. A: 3 representative image of untreated WT fibroblasts, 3 representative images of untreated NPC fibroblasts, and 3 representative images of NPC fibroblasts treated with Rp-cAMPS (50 μM) for 48 h. Fibroblasts are stained for free cholesterol (filipin). Images are representative of at least 25 images for each condition taken over 5 separate experiments. B: representative gel showing βarr2 and actin expression in WT and NPC fibroblasts and densitometry analysis of βarr2 protein expression normalized to actin content. Significance was determined by t-test (n = 3; *P = 0.04).