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

Fredrick L Dunn, Scott M Grundy, David W. Bilheimer, Richard J. Havel, Philip Raskin

Research output: Contribution to journalArticle

14 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)316-324
Number of pages9
JournalMetabolism
Volume34
Issue number4
DOIs
StatePublished - 1985

Fingerprint

Hypertriglyceridemia
Apolipoproteins E
LDL Lipoproteins
Apolipoproteins C
very low density lipoprotein triglyceride
Apoproteins
Glycerol
Obesity
Phenotype

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

Cite this

Impaired catabolism of very low-density lipoprotein-triglyceride in a family with primary hypertriglyceridemia. / Dunn, Fredrick L; Grundy, Scott M; Bilheimer, David W.; Havel, Richard J.; Raskin, Philip.

In: Metabolism, Vol. 34, No. 4, 1985, p. 316-324.

Research output: Contribution to journalArticle

Dunn, Fredrick L ; Grundy, Scott M ; Bilheimer, David W. ; Havel, Richard J. ; Raskin, Philip. / Impaired catabolism of very low-density lipoprotein-triglyceride in a family with primary hypertriglyceridemia. In: Metabolism. 1985 ; Vol. 34, No. 4. pp. 316-324.
@article{b024e987c7f3486492bc4610c5e12747,
title = "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.",
author = "Dunn, {Fredrick L} and Grundy, {Scott M} and Bilheimer, {David W.} and Havel, {Richard J.} and Philip Raskin",
year = "1985",
doi = "10.1016/0026-0495(85)90220-3",
language = "English (US)",
volume = "34",
pages = "316--324",
journal = "Metabolism: Clinical and Experimental",
issn = "0026-0495",
publisher = "W.B. Saunders Ltd",
number = "4",

}

TY - JOUR

T1 - Impaired catabolism of very low-density lipoprotein-triglyceride in a family with primary hypertriglyceridemia

AU - Dunn, Fredrick L

AU - Grundy, Scott M

AU - Bilheimer, David W.

AU - Havel, Richard J.

AU - Raskin, Philip

PY - 1985

Y1 - 1985

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0021861560&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0021861560&partnerID=8YFLogxK

U2 - 10.1016/0026-0495(85)90220-3

DO - 10.1016/0026-0495(85)90220-3

M3 - Article

C2 - 3884962

AN - SCOPUS:0021861560

VL - 34

SP - 316

EP - 324

JO - Metabolism: Clinical and Experimental

JF - Metabolism: Clinical and Experimental

SN - 0026-0495

IS - 4

ER -