Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts.
L Liscum, JR Faust - Journal of Biological Chemistry, 1987 - ASBMB
One characteristic of type C Niemann-Pick (NPC) disease is the substantial intracellular
accumulation of unesterified cholesterol. The increased cholesterol content in NPC
fibroblasts which are grown in the presence of low density lipoproteins (LDL) has been
postulated to be due to a deficiency in cellular cholesterol esterification. We have examined
several aspects of LDL metabolism in NPC fibroblasts. We observe that LDL binding,
internalization, and lysosomal hydrolysis of LDL cholesteryl esters are normal in NPC cells …
accumulation of unesterified cholesterol. The increased cholesterol content in NPC
fibroblasts which are grown in the presence of low density lipoproteins (LDL) has been
postulated to be due to a deficiency in cellular cholesterol esterification. We have examined
several aspects of LDL metabolism in NPC fibroblasts. We observe that LDL binding,
internalization, and lysosomal hydrolysis of LDL cholesteryl esters are normal in NPC cells …