alexa Plasma metabolism of apoB-containing lipoproteins in patients with hepatic lipase deficiency.
Pharmaceutical Sciences

Pharmaceutical Sciences

Journal of Clinical & Experimental Pharmacology

Author(s): Ruel IL, Couture P, Cohn JS, Lamarche B, Ruel IL, Couture P, Cohn JS, Lamarche B

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Abstract The metabolism of apoB-containing lipoproteins was investigated in the fasted state in three complete and three partial hepatic lipase (HL)-deficient subjects as well as in seven normotriglyceridemic (NTG) and two hypertriglyceridemic (HTG) controls using a 12 h primed-constant infusion of L-[5,5,5-D(3)]-leucine. Two males with complete HL deficiency had increased plasma pool sizes of VLDL and IDL apoB-100 due to substantial reductions in fractional catabolic rate (FCR) of VLDL and IDL apoB-100 compared with both NTG and HTG controls. Reductions in LDL apoB-100 production rate (PR) were also observed in these two patients compared with NTG and HTG controls. Complete HL deficiency in the female proband was associated with normal VLDL apoB-100 kinetics, while plasma IDL apoB-100 pool size was increased by 124\% due to an 82\% decrease in the FCR of IDL apoB-100. The FCR and PR of LDL apoB-100 were reduced by 64 and 51\%, respectively, in the proband compared with sex-matched controls. Partial HL-deficient patients were characterized by apoB-containing lipoprotein metabolism similar to that of controls. These results indicate that complete HL deficiency is associated with a potentially atherogenic apoB-containing lipoprotein metabolism that can be modulated considerably by secondary factors such as gender and abdominal obesity. This article was published in Atherosclerosis and referenced in Journal of Clinical & Experimental Pharmacology

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