Impaired catabolism of very low-density lipoprotein-triglyceride in a family with primary hypertriglyceridemia.

Abstract

In this report, kinetic studies of plasma very low-density lipoprotein-triglyceride (VLDL-TG) were examined in five brothers (three affected and two unaffected) from a family with primary hypertriglyceridemia. Synthesis and catabolism of VLDL-TG were studied by in vivo labelling of plasma TG with 3H-glycerol, and multicompartmental analysis of the plasma die-away curves. Results of the kinetic studies revealed the following information: (1) one brother, who had the highest plasma TG level and was obese, had both overproduction and a reduced fractional catabolic rate (FCR) of VLDL-TG; (2) second brother, who had moderate hypertriglyceridemia, had a low FCR and high-normal synthesis of VLDL-TG; (3) a third, who had only mildly elevated TG, had a low FCR and normal synthesis of VLDL-TG; and (4) the two normolipidemic brothers had neither overproduction nor decreased FCR of VLDL-TG. The composition of the soluble apoproteins of VLDL was normal. The apoprotein E phenotypes were E4/3 in four brothers, and E3/2 in the fifth. We have reached the following conclusions regarding this family: (1) the common kinetic abnormality of VLDL-TG metabolism in the hypertriglyceridemic brothers was a low clearance of VLDL-TG; (2) impaired catabolism of VLDL could not be explained by the apoprotein C or E patterns; and (3) the most severe hypertriglyceridemia occurred when the decreased FCR was present in conjunction with VLDL-TG overproduction due to obesity. Thus, a moderate defect in catabolism of plasma TG appears to be responsible for one familial form of primary hypertriglyceridemia.

• Authors: F L Dunn, S M Grundy, D W Bilheimer, R J Havel, P Raskin
• Journal: Metabolism: clinical and experimental (Metabolism) Vol. 34 Issue 4 Pg. 316-24 (Apr 1985) ISSN: 0026-0495 [Print] United States
• PMID: 3884962 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Apolipoproteins
• Insulin
• Lipids
• Lipoproteins
• Lipoproteins, VLDL
• Triglycerides
• very low density lipoprotein triglyceride

Topics

• Adult
• Apolipoproteins (blood)
• Biological Transport
• Glucose Tolerance Test
• Humans
• Hyperlipoproteinemia Type IV (blood, genetics)
• Insulin (blood)
• Kinetics
• Lipids (blood)
• Lipoproteins (blood)
• Lipoproteins, VLDL (blood)
• Male
• Middle Aged
• Pedigree
• Triglycerides (blood)