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(Arteriosclerosis, Thrombosis, and Vascular Biology. 2000;20:1796.)
© 2000 American Heart Association, Inc.

Atherosclerosis and Lipoproteins

Increased Production of HDL ApoA-I in Homozygous Familial Defective ApoB-100

J. R. Schaefer; K. Winkler; H. Schweer; M. M. Hoffmann; M. Soufi; H. Scharnagl; B. Maisch; H. Wieland; A. Steinmetz; W. März

From the Department of Internal Medicine (J.R.S., M.S., B.M., A.S.), Division of Cardiology, University of Marburg. Marburg; the Department of Medicine (K.W., M.M.H., H.S., H.W., W.M.), Division of Clinical Chemistry, University of Freiburg, Freiburg; and Children’s Hospital (H.S.), University of Marburg, Marburg, Germany.

Correspondence to Dr Winfried März, Department of Medicine, Division of Clinical Chemistry, Hugstetter Strasse 55, 79106 Freiburg, Germany. E-mail maerz{at}med1.ukl.uni-freiburg.de

Abstract—Familial defective apolipoprotein (apo) B-100 (FDB) is a frequent cause of hypercholesterolemia. Hypercholesterolemia in homozygous FDB is less severe than in homozygotes for familial hypercholesterolemia. Recently, we showed decreased low density lipoprotein (LDL) apoB-100 fractional catabolism and decreased production of LDL due to an enhanced removal of apoE-containing precursors in a patient with homozygous FDB. The effects of defective apoB-100 on high density lipoprotein (HDL) metabolism are unknown. We studied HDL apoA-I metabolism in this FDB patient and in 6 control subjects by using ²H₃-L-leucine as a tracer. ApoA-I levels were normal in all study subjects. However, the fractional catabolic rate and the production rate of apoA-I were increased, by 79% and 70%, respectively, in FDB; the fractional catabolic rate of apoA-I in FDB was 0.34 day^-1 compared with 0.19±0.03 day^-1 in normal controls. The production rate of apoA-I in FDB was 18.4 mg · kg^-1 · d^-1 compared with 10.8±2.3 mg · kg^-1 · d^-1 in controls. Thus, we have shown for the first time that defective apoB-100 may influence HDL kinetics. The increase in total HDL turnover might enhance reverse cholesterol transport and could contribute to the seemingly benign clinical course of FDB compared with that of familial hypercholesterolemia.

Key Words: hypercholesterolemia • reverse cholesterol transport • tracer kinetics • stable isotopes

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