Lipoprotein abnormalities associated with a familial deficiency of hepatic lipase

Breckenridge, W. C.; Little, J. A.; Alaupovic, Petar; Wang, C.S; Kuksis, A.; Kakis, G.; Lindgren, Frank T.; Gardiner, Geoffrey W.

doi:10.1016/0021-9150(82)90136-8

Cited by 312 publications

(112 citation statements)

References 47 publications

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“…Using a nonhuman primate model, Goldberg et al [2] achieved complete inhibition of circulating HL activity and demonstrated that accumulation of VLDL, IDL and lesser density LDL in plasma occurred in association with an impaired conversion of radiolabeled VLDL to IDL and LDL. In some studies in the rat analogous results have been obtained [3,4], Familial hepatic lipase deficiency is also characterized by accumulation of TG-rich lipoprotein fractions in plasma [24].…”

Section: Discussionsupporting

confidence: 62%

Very low density lipoprotein triglyceride kinetics during hepatic lipase suppression by estrogen

et al. 1985

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The exact role of the heparin-releasable hepatic endothelial lipase has remained controversial. It has been suggested that it acts in concert with lipoprotein lipase in the step-wise delipidation of triglyceride-rich lipoproteins. On the other hand, there is evidence indicating that high density lipoprotein, is the preferred substrate for hepatic lipase. Here, it is shown that a moderate (27%) suppression of hepatic lipase activity by estrogen did not impair removal of 3H-labeled very low density lipoproteins (VLDL) triglycerides, suggesting that this enzyme is not a major regulator of VLDL catabolism under physiological circumstances. Lipoprotein Hepatic lipase Lipoprotein lipase

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Section: Discussionsupporting

confidence: 62%

Very low density lipoprotein triglyceride kinetics during hepatic lipase suppression by estrogen

et al. 1985

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“…Tel: 82-2-760-2684; Fax: 82-2-762-9662 E-mail: ihchae@snu.ac.kr (Lamarche et al, 1997), have pointed towards the proatherogenic role of hepatic lipase. However, reports that patients with the hepatic lipase deficiency developed premature CAD (Breckenridge et al, 1982) and animal studies, which showed that the hepatic lipase transgenic mice have less aortic cholesterol deposition (Busch et al, 1994), have pointed towards the antiatherogenic role of hepatic lipase.…”

Section: Introductionmentioning

confidence: 99%

Hepatic Lipase C514T Polymorphism and its Relationship with Plasma HDL-C Levels and Coronary Artery Disease in Koreans

Park¹,

Choi²,

Chae³

et al. 2003

BMB Reports

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“…The discovery of familial HL deficiency 13 has provided an opportunity to investigate the role of HL in lipoprotein metabolism in humans. The proband and his brother were markedly hvpertriglyceridemic, with hyperprebetalipoproteinemia, /3-VLDL, normal or elevated concentrations of LDL cholesterol and HDL cholesterol, and elevated concentrations of LDL and HDL triglyceride and phospholipid.…”

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confidence: 99%

“…Recently another family with HL deficiency and virtually identical qualitative lipoprotein abnormalities has been described. 14 The brothers studied in our laboratory have significant ischemic vascular disease and plasma apolipoprotein (apo) B levels approximately twice the normal level, 13 in contrast to the Scandinavian subjects, 14 who had no apparent ischemic vascular disease and normal levels of plasma apo B. The turnover of apo B has been studied in one of the Scandinavian subjects, and it was found that the conversion of VLDL to IDL was reduced by 50%, and the conversion of IDL to LDL was reduced to 5% of the control values.…”

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confidence: 99%

“…15 It was also reported that by rate zonal ultracentrifugation there was no LDL 15 This is in contrast to the subjects reported by this laboratory in whom LDL was identified in significant quantities by preparative and analytical ultracentrifugatjon. 13 /3-VLDL is also a characteristic of dysbetalipoproteinemia. 16 0-VLDL are cholesterol-rich remnants of VLDL and chylomicron metabolism that accumulate in dysbetalipoproteinemia because of the presence of the poorly functional form of apo E, apo E2.…”

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confidence: 99%

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Plasma lipoproteins in familial hepatic lipase deficiency.

et al. 1990

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We have studied the lipoproteins, apolipoproteins, and postheparin lipase activities In an extended pedigree with familial hepatic lipase deficiency. A deficiency of hepatic lipase was found In three of five brothers and In one of their children. Trlglycerlde enrichment of low density and high density lipoproteins was Identified as the constitutive phenotype. 0-very low density lipoproteln was observed In hepatic llpase-deflclent subjects, but It was absent when the plasma trlglycerlde concentration was less than 1 mM/l. The hepatic llpase-deflclent subjects had normal or elevated low density lipoproteln cholesterol and high density lipoproteln cholesterol concentrations. Hyperprebetalipoprotelnemla, hyperbetallpoprotelnemla, and hyperalphallpoproteinemla were observed In both affected and unaffected family members. Compared with the unaffected family members, the hepatic lipase-deflclent subjects had no significant differences In very low density lipoproteln cholesterol, very low density lipoproteln trlglycerlde, or low density lipoproteln cholesterol. These observations are consistent with the presence of additional genes causing hyperilpIdemla In this family, independent of the deficiency of hepatic lipase. The role of HL in lipoprotein metabolism is only partly understood. 1 -» Postheparin plasma HL activity has been reported to be positively correlated with VLDL concentration and negatively correlated with HDL cholesterol concentration.2 -5 It is known that in vitro HL will hydrolyze HDL triglyceride and phospholipid. 6 Intermediate density lipoproteins (IDL) and low density lipoproteins (LDL) with a density between 1.019 and 1.045 have also been shown to be in vitro substrates of HL. 78 The inhibition of HL in vivo by infusion of anti-HL antibody into rats 9 ' 1011 and cynomolgous monkeys 12 has been found to result in the increase of HDL and IDL. However, there is no agreement on the temporal relationships of these changes and no consistent changes in IDL between species. In the study on the primate model, 12 VLDL triglyceride concentrations increased 60% to 300% after infusion of anti-HL antibody. These authors did not report whether the VLDL contained particles of /3 electrophoretic mobility.The discovery of familial HL deficiency 13 has provided an opportunity to investigate the role of HL in lipoprotein metabolism in humans. The proband and his brother were markedly hvpertriglyceridemic, with hyperprebetalipoproteinemia, /3-VLDL, normal or elevated concentrations of LDL cholesterol and HDL cholesterol, and elevated concentrations of LDL and HDL triglyceride and phospholipid. Recently another family with HL deficiency and virtually identical qualitative lipoprotein abnormalities has been described.14 The brothers studied in our laboratory have significant ischemic vascular disease and plasma apolipoprotein (apo) B levels approximately twice the normal level, 13 in contrast to the Scandinavian subjects, 14 who had no apparent ischemic vascular disease and normal levels of plasma apo B. The turnover of ...

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Lipoprotein abnormalities associated with a familial deficiency of hepatic lipase

Cited by 312 publications

References 47 publications

Very low density lipoprotein triglyceride kinetics during hepatic lipase suppression by estrogen

Very low density lipoprotein triglyceride kinetics during hepatic lipase suppression by estrogen

Hepatic Lipase C514T Polymorphism and its Relationship with Plasma HDL-C Levels and Coronary Artery Disease in Koreans

Plasma lipoproteins in familial hepatic lipase deficiency.

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