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Characterization of cholesterol metabolism and apolipoprotein E glycosylation in the liver of a Niemann‐Pick Type C1 animal model
Author(s) -
LIM MeiLi,
CHUA ChingChing,
HO Jacqueline ZyetEe,
WONG BoonSeng
Publication year - 2008
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.22.1_supplement.823.15
Subject(s) - apolipoprotein e , npc1 , cholesterol , liver x receptor , lipoprotein , reverse cholesterol transport , biology , apolipoprotein b , endocrinology , medicine , ldl receptor , homeostasis , chemistry , endosome , biochemistry , receptor , nuclear receptor , disease , gene , transcription factor
Niemann‐Pick type C1 (NPC1) disease is a cholesterol‐storage disorder characterized by liver dysfunction. NPC1 is highly expressed in the liver and the protein regulates the transport of cholesterol from late endosomes/lysosomes to other cellular compartments involved in intracellular cholesterol homeostasis. Another major protein involved in cholesterol metabolism is apolipoprotein E (ApoE). ApoE is also highly expressed in the liver and function as a major component of lipoprotein in cholesterol homeostasis. Altered ApoE expression is associated with defective low‐density lipoprotein receptor (LDL‐R) and may cause hypercholesterolemia. Earlier studies have shown that ApoE is differentially sialylated. However, the functional significance of this chemical alteration on ApoE and its association with cholesterol homeostasis is unknown. In this study, we will present the correlation between the glycan profile on hepatic ApoE and cholesterol metabolism at early and late stages of pathology in a NPC1 animal model. In addition, we will show the connection between changes in cholesterol metabolism with the biochemical profile of high‐density lipoprotein (HDL), LDL and triglycerides at different stages of disease development in the liver.