[HTML][HTML] NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols
A Frolov, SE Zielinski, JR Crowley… - Journal of Biological …, 2003 - ASBMB
Mutations in the Niemann-Pick disease genes cause lysosomal cholesterol accumulation
and impaired low density lipoprotein (LDL) cholesterol esterification. These findings have
been attributed to a block in cholesterol movement from lysosomes to the site of the sterol
regulatory machinery. In this study we show that Niemann-Pick type C1 (NPC1) and
Niemann-Pick type C2 (NPC2) mutants have increased cellular cholesterol, yet they are
unable to suppress LDL receptor activity and cholesterol biosynthesis. Cholesterol overload …
and impaired low density lipoprotein (LDL) cholesterol esterification. These findings have
been attributed to a block in cholesterol movement from lysosomes to the site of the sterol
regulatory machinery. In this study we show that Niemann-Pick type C1 (NPC1) and
Niemann-Pick type C2 (NPC2) mutants have increased cellular cholesterol, yet they are
unable to suppress LDL receptor activity and cholesterol biosynthesis. Cholesterol overload …