Complete deficiency of the low-density lipoprotein receptor is associated with increased apolipoprotein B-100 production.
John S Millar, Cyrille Maugeais, Katsunori Ikewaki, Daniel M Kolansky, P Hugh R Barrett, Elaine C Budreck, Raymond C Boston, Norio Tada, Seibu Mochizuki, Joep C Defesche, James M Wilson, Daniel J Rader
Arterioscler Thromb Vasc Biol | Published : 2005
OBJECTIVE: We addressed the role of the low-density lipoprotein (LDL) receptor in determining clearance rates and production rate (PR) of apolipoprotein B (apoB) in humans. METHODS AND RESULTS: Kinetic studies using endogenous labeling of apoB with deuterated leucine were performed in 7 genetically defined patients with homozygous familial hypercholesterolemia (FH) and compared with 4 controls. The fractional catabolic rates (FCR) and PRs for apoB were determined by multicompartmental modeling. The FCRs of very-low-density lipoprotein 1 (VLDL1), VLDL2, intermediate-density lipoprotein (IDL), and LDL apoB were lower in FH than in controls, with the LDL apoB FCR being significantly lower (0.14..View full abstract
Awarded by NHLBI NIH HHS
Awarded by NCRR NIH HHS
Awarded by NIBIB NIH HHS