[HTML][HTML] Correction of apolipoprotein AI-mediated lipid efflux and high density lipoprotein particle formation in human Niemann-Pick type C disease fibroblasts
E Boadu, HY Choi, DWK Lee, EI Waddington… - Journal of biological …, 2006 - ASBMB
Impaired cell cholesterol trafficking in Niemann-Pick type C (NPC) disease results in the first
known instance of impaired regulation of the ATP-binding cassette transporter A1 (ABCA1),
a lipid transporter mediating the rate-limiting step in high density lipoprotein (HDL)
formation, as a cause of low plasma HDL-cholesterol in humans. We show here that
treatment of human NPC1-/-fibroblasts with the liver X receptor (LXR) agonist TO-901317
increases ABCA1 expression and activity in human NPC1-/-fibroblasts, as indicated by near …
known instance of impaired regulation of the ATP-binding cassette transporter A1 (ABCA1),
a lipid transporter mediating the rate-limiting step in high density lipoprotein (HDL)
formation, as a cause of low plasma HDL-cholesterol in humans. We show here that
treatment of human NPC1-/-fibroblasts with the liver X receptor (LXR) agonist TO-901317
increases ABCA1 expression and activity in human NPC1-/-fibroblasts, as indicated by near …