title: Plasma metabolism of apoB-containing lipoproteins in patients with hepatic lipase deficiency.
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.
authors.affiliation: Institute on Nutraceuticals and Functional Foods, Pavillon INAF, Local 2742, 2440 Hochelaga Blvd., Laval University, Que., Canada G1K 7P4.
authors.forename: Isabelle L
authors.initials: IL
authors.lastname: Ruel
authors.forename: Patrick
authors.initials: P
authors.lastname: Couture
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Cohn
authors.forename: Benoît
authors.initials: B
authors.lastname: Lamarche
mesh_terms: D001055:Apolipoproteins B / Q000378:metabolism*; D001161:Arteriosclerosis / Q000235:genetics* / Q000503:physiopathology*; D016022:Case-Control Studies; D008078:Cholesterol, LDL / Q000097:blood; D005260:Female; D006801:Humans; D007700:Kinetics; D008049:Lipase / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism; D008297:Male; D009765:Obesity / Q000150:complications; D012737:Sex Factors
chemical_list: D001055:Apolipoproteins B; D008078:Cholesterol, LDL; C504173:LIPC protein, human; D008049:Lipase