Increased hepatic secretion of very-low-density lipoprotein apolipoprotein B-100 in cholesteryl ester storage disease.
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.
Cummings
M H
MH
Department of Medicine, St. Thomas' Hospital (UMDS), London, UK.
Watts
G F
GF
D000328:Adult; D053299:Apolipoprotein B-100; D001055:Apolipoproteins B / Q000378:metabolism*; D002784:Cholesterol / Q000097:blood; D015217:Cholesterol Ester Storage Disease / Q000503:physiopathology*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D008079:Lipoproteins, VLDL / Q000378:metabolism*; D008099:Liver / Q000378:metabolism*; D008297:Male; D008798:Mevalonic Acid / Q000097:blood; D014280:Triglycerides / Q000097:blood
D053299:Apolipoprotein B-100; D001055:Apolipoproteins B; D008079:Lipoproteins, VLDL; D014280:Triglycerides; D002784:Cholesterol; D008798:Mevalonic Acid