[HTML][HTML] Impaired ABCA1-dependent lipid efflux and hypoalphalipoproteinemia in human Niemann-Pick type C disease
HY Choi, B Karten, T Chan, JE Vance, WL Greer… - Journal of Biological …, 2003 - ASBMB
The cholesterol trafficking defect in Niemann-Pick type C (NPC) disease leads to impaired
regulation of cholesterol esterification, cholesterol synthesis, and low density lipoprotein
receptor activity. The ATP-binding cassette transporter A1 (ABCA1), which mediates the rate-
limiting step in high density lipoprotein (HDL) particle formation, is also regulated by cell
cholesterol content. To determine whether the Niemann-Pick C1 protein alters the
expression and activity of ABCA1, we determined the ability of apolipoprotein AI (apoA-I) to …
regulation of cholesterol esterification, cholesterol synthesis, and low density lipoprotein
receptor activity. The ATP-binding cassette transporter A1 (ABCA1), which mediates the rate-
limiting step in high density lipoprotein (HDL) particle formation, is also regulated by cell
cholesterol content. To determine whether the Niemann-Pick C1 protein alters the
expression and activity of ABCA1, we determined the ability of apolipoprotein AI (apoA-I) to …