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
N1 - Funding Information:
From the Department of Internal Medicine and Center for Human Nutrition, University of Texas Health Science Center at Dallas, Southwestern Medical School, Dallas, Tex; and the Cardiovascular Research Institute and Department of Medicine, University of California. San Francisco. Supported by NIH Grants AM 18179, HL15949. I-MOl-RR0063. AM 16667, H6l4197, the Veterans Administration, and the American Heart Association. Dr Dunn was a recipient of a fellowship award from the Juvenile Diabetes Foundation. This study was presented in part at the National Meeting of the American Federation for Clinical Research, April, 1983. Address reprint requests to Fredrick L. Dunn, MD, Joslin Diabetes Center, One Joslin Place. Boston, MA 02215. 0 1985 by Grune & Stratton, Inc. 0026-0495/85/3404-0003$3.00/O
PY - 1985/4
Y1 - 1985/4
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.
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U2 - 10.1016/0026-0495(85)90220-3
DO - 10.1016/0026-0495(85)90220-3
M3 - Article
C2 - 3884962
AN - SCOPUS:0021861560
SN - 0026-0495
VL - 34
SP - 316
EP - 324
JO - Metabolism
JF - Metabolism
IS - 4
ER -