Plasma lipoprotein abnormalities associated with acquired hepatic triglyceride lipase deficiency

Goldberg, Ira J.; Mazlen, Roger G.; Rubenstein, Ardon; Gibson, J. B.; Paterniti, James R.; Lindgren, Frank T.; Brown, W. Virgil

doi:10.1016/0026-0495(85)90107-6

Cited by 31 publications

(9 citation statements)

References 20 publications

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“…The patient with HL deficiency had an accumulation of a TG-enriched, large and buoyant apo B containing lipoproteins as determined by DGUC, SVS-apo B, SDS-PAGGE, and nondenaturing GGE. These observations correspond with previous studies that have shown the accumulation of TG-rich lipoproteins and slightly more buoyant 'LDL' in both familial 1011 and acquired 15 forms of HL deficiency as described by analytical uitracentrtfugation. Triglycerideenriched 'LDL' from HL-deficient subjects differs dramatically from dense LDL isolated from subjects with hypertriglyceridemia.…”

Section: Discussionsupporting

confidence: 92%

Large buoyant LDL-like particles in hepatic lipase deficiency.

et al. 1989

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Hepatic lipase (HL) Is thought to play a role In processing very low density llpoproteln to low density llpoproteln (LDL). To analyze the relationship between HL and LDL, the density, size, and chemical composition of LDL Isolated from 18 normal subjects and from three subjects with reduced or absent levels of HL activity were compared. In an HL-deficlent subject the major peak of apoproteln (apo) Bcontalning llpoprotelns ('LDL') had a density of 1.023 g/ml and a diameter of 26.4 nm compared to male control subjects (1.044±0.006 g/ml and 25.3±0.3 nm). Two half-sisters of the HL-deflclent subject with half the normal levels of HL activity had LDL that also were more buoyant and slightly larger than the LDL Isolated from female control subjects. The peak density and average diameter of LDL were correlated with HL activity, consistent with the hypothesis that HL Influenced formation and physical characteristics of typical LDL. Apo B-100 was the major apoproteln In the 'LDL' isolated from the HL-deflclent subject and contained a greater proportion of trlglycerlde compared to the control subjects' LDL. The absence of HL appears to prevent the production of classical LDL Our data support the hypothesis that HL helps determine normal LDL characteristics. . A complicated picture of a 'delipidation cascade' is emerging in which the factors that determine whether VLDL will be removed from the circulation or undergo conversion into LDL is just being delineated. Several intravascular enzymes are thought to be involved in the formation of LDL, including lipoprotein lipase (LPL), llpid transfer protein, and hepatic triglyceride lipase (HL). Rubinstein et al. 3 have hypothesized that HL is involved in the conversion of IDL to LDL; however, direct experimental data describing the mechanisms involved in this conversion are, as yet, undefined. 34The conversion of VLDL remnants or IDL to LDL is thought to occur across the splanchnic bed where HL is located.35 Support for a role of HL among the factors involved in the processing of IDL to LDL has been derived from observations made on humans and animals with HL deficiency; humans wtth diseases associated with reduced levels of HL activity (hypothyroidism, uremia, chronic liver disease); and in experimentally induced HL deficiency in rats and monkeys. 11 -15 In these studies, reduced levels of HL were associated with an increase in IDL concentration and TG accumulation in LDL.6 - 14The present study was designed to examine the possible relationships between HL activity and LDL physical characteristics in normolipidemic subjects and in subjects with reduced or absent HL activity levels. Methods Study SubjectsEighteen healthy Caucasian subjects (nine women and nine men), with a mean age of 35.4±11.3 years for women and 26.9±5.6 years for men (range, 18 to 48 years), were recruited for this study, and they participated on a voluntary basis. None of these subjects took any medication or had evidence of a lipoprotein disorder. All patients belonged to a single family with HL defic...

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Section: Discussionsupporting

confidence: 92%

Large buoyant LDL-like particles in hepatic lipase deficiency.

et al. 1989

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“…The changes in LDL size were accounted for by differences in HL activity to the extent of 26% in CAD patients and 36% in normolipi- demic subjects. These data confirm previous studies that showed the presence of more buoyant and larger LDLs in both familial 10 " and acquired 12 forms of HL deficiency. The low coefficients of correlation (r) obtained for the association between HL activity and LDL size in both populations indicate that other factors, genetically and environmentally modulated, 262733 also play an important role in accounting for changes in LDL size.…”

Section: Discussionsupporting

confidence: 82%

Effect of hepatic lipase on LDL in normal men and those with coronary artery disease.

Zambón

Austin

Brown

et al. 1993

Arterioscler Thromb

234

117

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Hepatic triglyceride lipase (HL) is thought to play a role in the formation of low density lipoproteins (LDLs) from small very low density lipoproteins (VLDLs) and intermediate density lipoproteins (IDLs).To analyze the possible physiological role of HL in determining LDL buoyancy, size, and chemical composition, HL activity and LDL were studied in 21 patients with coronary artery disease (CAD) and 23 normolipidemic subjects. In both groups, LDL buoyancy and size were inversely associated with HL activity levels. The effect of HL on LDL size was comparable in CAD patients and in normolipidemic subjects. HL appeared to influence LDL lipid composition primarily by affecting the surface lipid components. The free cholesterol content of LDL particles was highly correlated with HL activity in both CAD and normolipidemic individuals. The free cholesterol to phospholipid ratio in LDL particles correlated with HL in both CAD and normolipidemic subjects. When the individuals were separated according to their LDL subclass patterns, pattern B subjects had significantly higher HL than pattern A subjects in both CAD and normolipidemic groups. LDL may take place. 9 The strongest evidence supporting the role of HL in LDL formation comes from studies performed on human and animal models with HL deficiency. -14 In these studies, low HL activity was associated with larger and more buoyant LDL particles, often extending into the IDL density range.To further examine the possible involvement of HL in determining the physical characteristics of LDL, the compositional and physical properties of LDL were examined in relation to HL activity levels in CAD patients and normolipidemic subjects. Methods SubjectsTwenty-one men aged 50.2 ±9 (mean±SEM) years and with elevated apolipoprotein (apo) B plasma levels (>125 mg/dl), a family history of CAD, and evidence of coronary atherosclerosis by coronary arteriography 3 were selected. All subjects were participants in a clinical intervention trial of intensive drug therapy for CAD; blood samples for the present study were drawn at a protocol time point 6 weeks after discontinuing lipidlowering therapy, at which time all LDL and apo B-related measurements had returned to pretreatment baseline levels.3 Two subjects were taking /J-blockers at the time of the present study. Dietary advice was given according to the American Heart Association's step I and II diets.3 Patients with diabetes; severe hypertension; cancer; or liver, thyroid, or kidney diseases were excluded.An additional 23 Caucasian men aged 33 ±2 years were recruited by advertisement and were paid to by guest on May 11, 2018 http://atvb.ahajournals.org/ Downloaded from

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“…Although HDL is inappropriately not decreased, the most striking lipoprotein phenotype is increased LDL/intermediatedensity lipoprotein triglyceride and cholesterol. 17,18 The effect of estrogen on LpL is controversial. Although Homma and colleagues 19 reported that estrogens can inhibit the LpL promoter activity, causing LpL deficiency, others have not found similar effects.…”

Section: Discussionmentioning

confidence: 99%

Hypertriglyceridemia-induced pancreatitis created by oral estrogen and in vitro fertilization ovulation induction

Lee

Goldberg

2008

Journal of Clinical Lipidology

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Hypertriglyceridemia is one of the known causes of pancreatitis. Estrogen treatment can aggravate hypertriglyceridemia by increasing very low density lipoprotein secretion and reducing hepatic triglyceride lipase. In this paper, we present 3 patients who developed severe hypertriglyceridemia with conditions that increased estrogen. Two patients were found to have genetic lipoprotein lipase deficiency and were treated with birth control pills. The third was a patient with polycystic ovary disease who was receiving ovulation induction therapy for in vitro fertilization.

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Plasma lipoprotein abnormalities associated with acquired hepatic triglyceride lipase deficiency

Cited by 31 publications

References 20 publications

Large buoyant LDL-like particles in hepatic lipase deficiency.

Large buoyant LDL-like particles in hepatic lipase deficiency.

Effect of hepatic lipase on LDL in normal men and those with coronary artery disease.

Hypertriglyceridemia-induced pancreatitis created by oral estrogen and in vitro fertilization ovulation induction

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