Increased hepatic secretion of very-low-density lipoprotein apolipoprotein B-100 in cholesteryl ester storage disease.

Abstract

Using a stable isotope method, we measured the hepatic secretion rate of very-low-density lipoprotein apolipoprotein B-100 (VLDL apoB) in a 26-year-old women who had dyslipidemia due to cholesteryl ester storage disease (CESD) and in five normolipidemic subjects. [1-13C]Leucine was administered by a primed constant intravenous infusion and the enrichment of VLDL apoB was determined by gas chromatography-mass spectrometry. The absolute secretion rate (ASR) of VLDL apoB in the patient was more than twice the mean ASR of the normolipidemic group (17.1 vs 8.0 +/- 0.8 mg/kg body wt. per day). The plasma mevalonic acid concentration, a measure of intrahepatic cholesterol synthesis, was also greater in the patient than in the normolipidemic subjects (8.3 vs 4.4 +/- 1.8 micrograms/L). The findings are consistent with the hypothesis that in CESD increased intrahepatic synthesis of cholesterol stimulates hepatic secretion of VLDL apoB and this may partly account for the dyslipidemia.

• Authors: M H Cummings, G F Watts
• Journal: Clinical chemistry (Clin Chem) Vol. 41 Issue 1 Pg. 111-4 (Jan 1995) ISSN: 0009-9147 [Print] England
• PMID: 7813057 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Apolipoprotein B-100
• Apolipoproteins B
• Lipoproteins, VLDL
• Triglycerides
• Cholesterol
• Mevalonic Acid

Topics

• Adult
• Apolipoprotein B-100
• Apolipoproteins B (metabolism)
• Cholesterol (blood)
• Cholesterol Ester Storage Disease (physiopathology)
• Female
• Gas Chromatography-Mass Spectrometry
• Humans
• Lipoproteins, VLDL (metabolism)
• Liver (metabolism)
• Male
• Mevalonic Acid (blood)
• Triglycerides (blood)