Abstract
In Niemann-Pick disease type C fibroblasts, the deficiency of cholesterol esterification has been reported. In this experiment, we detected the attenuated elevation of cytoplasmic calcium concentration following low density lipoprotein uptake in the fibroblasts. Moreover, we administered calcium channel agonist ( 0.5 μM YC-170) and calcium channel antagonists to the fibroblasts. YC-170 improved the attenuated elevation of calcium concentration and the deficient cholesterol esterification by 40% and 90% respectively. Calcium channel antagonists decreased the cholesterol esterification in normal and affected fibroblasts. These data indicated that the attenuated elevation of cytoplasmic calcium concentration was strongly related to the etiology of this disease.
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