[HTML][HTML] Posttranslational regulation of acid sphingomyelinase in niemann-pick type C1 fibroblasts and free cholesterol-enriched chinese hamster ovary cells
JW Reagan, ML Hubbert, GS Shelness - Journal of Biological Chemistry, 2000 - ASBMB
Niemann-Pick type C disease is characterized by the accumulation of cholesterol and other
lipids within the lysosomal compartment, a process that is often accompanied by a reduction
in acid sphingomyelinase activity. These studies demonstrate that a CHO cell mutant (CT-
60), which accumulates lysosomal cholesterol because of a defective NP-C1 protein, has
approximately 5–10% of the acid sphingomyelinase activity of its parental cell line (25-RA)
or wild type (CHO-K1) cells. The cholesterol-induced reduction in acid sphingomyelinase …
lipids within the lysosomal compartment, a process that is often accompanied by a reduction
in acid sphingomyelinase activity. These studies demonstrate that a CHO cell mutant (CT-
60), which accumulates lysosomal cholesterol because of a defective NP-C1 protein, has
approximately 5–10% of the acid sphingomyelinase activity of its parental cell line (25-RA)
or wild type (CHO-K1) cells. The cholesterol-induced reduction in acid sphingomyelinase …